{ "schema_version": "2.0.0", "release_id": "human-metabolism-r97-map-v2", "title": "Human metabolism — an explorable reaction field", "scope": "A release-pinned, source-linked projection of selected human metabolic pathways spanning carbohydrate, energy, lipid, amino-acid, nucleotide, cofactor, porphyrin and biological-oxidation chemistry.", "caveat": "The map shows curated reaction direction and compartment identity, not measured flux, concentration, tissue dominance or thermodynamic magnitude. 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metabolism while preserving nitrogen handling as a separate question.", "color": "#77d68d", "x": 6310, "y": 1360, "width": 1940, "height": 1160, "pathway_ids": [ "branched-chain", "glutamate-glutamine", "phenylalanine-tyrosine", "tryptophan" ] }, { "id": "nitrogen-one-carbon", "label": "Nitrogen, one-carbon \u0026 thiols", "short_label": "NITROGEN + C1", "description": "Nitrogen disposal, one-carbon transfer, sulfur chemistry and redox-buffer synthesis at the amino-acid–nucleotide interface.", "color": "#a5e86f", "x": 100, "y": 2630, "width": 1940, "height": 1160, "pathway_ids": [ "serine", "sulfur-amino-acids", "glycine-glyoxylate", "urea-cycle", "glutathione" ] }, { "id": "nucleotides", "label": "Nucleotide economy", "short_label": "NUCLEOTIDES", "description": "Construction, interconversion, salvage and breakdown of purine and pyrimidine nucleotides.", "color": "#5fc5ff", "x": 2170, "y": 2630, "width": 1940, "height": 1160, "pathway_ids": [ "nucleotide-biosynthesis", 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"galactose", "label": "Galactose catabolism", "reactome_id": "R-HSA-70370", "domain_id": "sugar-branches-glycans", "description": "The Leloir-route chemistry that converts galactose-derived carbon into glucose-phosphate intermediates.", "color": "#6fd7ff", "x": 3165, "y": 215, "width": 870, "height": 447, "event_count": 5, "source_url": "https://reactome.org/content/detail/R-HSA-70370" }, { "id": "glycosaminoglycans", "label": "Glycosaminoglycan metabolism", "reactome_id": "R-HSA-1630316", "domain_id": "sugar-branches-glycans", "description": "Biosynthesis and turnover of sulfated and non-sulfated glycosaminoglycan chains across secretory and lysosomal compartments.", "color": "#6fd7ff", "x": 2225, "y": 694, "width": 870, "height": 447, "event_count": 10, "source_url": "https://reactome.org/content/detail/R-HSA-1630316" }, { "id": "oligosaccharide-recovery", "label": "Lysosomal oligosaccharide catabolism", "reactome_id": "R-HSA-8853383", "domain_id": "sugar-branches-glycans", 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"event_count": 12, "source_url": "https://reactome.org/content/detail/R-HSA-71403" }, { "id": "electron-transport", "label": "Respiratory electron transport", "reactome_id": "R-HSA-611105", "domain_id": "central-energy", "description": "Transfer of electrons through mitochondrial respiratory complexes and mobile carriers.", "color": "#ffb45d", "x": 4295, "y": 694, "width": 870, "height": 447, "event_count": 10, "source_url": "https://reactome.org/content/detail/R-HSA-611105" }, { "id": "chemiosmosis", "label": "ATP by chemiosmotic coupling", "reactome_id": "R-HSA-163210", "domain_id": "central-energy", "description": "ATP synthase chemistry driven by the electrochemical proton gradient across the inner mitochondrial membrane.", "color": "#ffb45d", "x": 5235, "y": 694, "width": 870, "height": 447, "event_count": 3, "source_url": "https://reactome.org/content/detail/R-HSA-163210" }, { "id": "malate-aspartate-shuttle", "label": "Malate–aspartate shuttle", "reactome_id": "R-HSA-9856872", "domain_id": "metabolic-integration", "description": "A coupled transport-and-reaction system that transfers cytosolic reducing equivalents into the mitochondrial matrix without moving NADH itself.", "color": "#f7d66c", "x": 6365, "y": 215, "width": 870, "height": 447, "event_count": 1, "source_url": "https://reactome.org/content/detail/R-HSA-9856872" }, { "id": "ketone-bodies", "label": "Ketone body metabolism", "reactome_id": "R-HSA-74182", "domain_id": "metabolic-integration", "description": "Hepatic formation and extrahepatic use of water-soluble acetyl-unit carriers.", "color": "#f7d66c", "x": 7305, "y": 215, "width": 870, "height": 447, "event_count": 10, "source_url": "https://reactome.org/content/detail/R-HSA-74182" }, { "id": "carbon-dioxide", "label": "Reversible CO₂ hydration", "reactome_id": "R-HSA-1475029", "domain_id": "metabolic-integration", "description": "Compartment-specific interconversion of carbon dioxide and hydrogencarbonate by carbonic anhydrases.", "color": 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"cholesterol", "label": "Cholesterol biosynthesis", "reactome_id": "R-HSA-191273", "domain_id": "complex-lipids", "description": "Multistep construction of cholesterol from acetyl-derived building blocks through isoprenoid and sterol intermediates.", "color": "#f58bc8", "x": 2225, "y": 1964, "width": 870, "height": 447, "event_count": 12, "source_url": "https://reactome.org/content/detail/R-HSA-191273" }, { "id": "triglycerides", "label": "Triglyceride metabolism", "reactome_id": "R-HSA-8979227", "domain_id": "complex-lipids", "description": "Assembly and hydrolysis of neutral-lipid stores and their exchange with glycerolipid intermediates.", "color": "#f58bc8", "x": 3165, "y": 1964, "width": 870, "height": 447, "event_count": 10, "source_url": "https://reactome.org/content/detail/R-HSA-8979227" }, { "id": "steroids", "label": "Steroid metabolism", "reactome_id": "R-HSA-8957322", "domain_id": "lipid-mediators", "description": "Interconversion of cholesterol-derived steroid frameworks 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the human Reactome event uses Q10. The terms are not silently collapsed.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-12462", "name": "Fumarase", "equation": "fumarate + H2O =\u003e (S)-malate", "source_id": "rhea", "source_record_ids": [ "RHEA:12462" ], "source_url": "https://www.rhea-db.org/rhea/12462", "rhea_parent_id": "RHEA:12460", "human_support": [ { "source": "Reactome", "id": "R-HSA-70982", "url": "https://reactome.org/content/detail/R-HSA-70982" } ], "route": "tca", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "Stereochemistry is preserved in the product name.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-21433", "name": "Malate dehydrogenase 2", "equation": "(S)-malate + NAD(+) =\u003e oxaloacetate + NADH + H(+)", "source_id": "rhea", "source_record_ids": [ "RHEA:21433" ], "source_url": "https://www.rhea-db.org/rhea/21433", "rhea_parent_id": "RHEA:21432", "human_support": [ { "source": "Reactome", "id": "R-HSA-70979", "url": "https://reactome.org/content/detail/R-HSA-70979" } ], "route": "tca", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "Human Reactome display direction; no thermodynamic or flux claim follows from the arrow.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "reactome-212651", "name": "Glutamine import — BlackBoxEvent", "equation": "", "source_id": "reactome", "source_record_ids": [ "R-HSA-212651" ], "source_url": "https://reactome.org/content/detail/R-HSA-212651", "rhea_parent_id": "", "human_support": [], "route": "transport", "compartment": "cytosol to mitochondrial matrix", "representation": "transport_black_box", "visual_style": "dotted", "direction_note": "A curated BlackBoxEvent, not a chemical reaction or passive-diffusion claim.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": true, "transport_species": [ "L-glutamine (cytosol)", "L-glutamine (mitochondrial matrix)" ], "qualifying_language": "" }, { "id": "rhea-15890", "name": "Glutaminase", "equation": "L-glutamine + H2O =\u003e L-glutamate + NH4(+)", "source_id": "rhea", "source_record_ids": [ "RHEA:15890" ], "source_url": "https://www.rhea-db.org/rhea/15890", "rhea_parent_id": "RHEA:15889", "human_support": [ { "source": "Reactome", "id": "R-HSA-70609", "url": "https://reactome.org/content/detail/R-HSA-70609" }, { "source": "UniProtKB", "id": "O94925", "url": "https://www.uniprot.org/uniprotkb/O94925/entry" }, { "source": "UniProtKB", "id": "Q9UI32", "url": "https://www.uniprot.org/uniprotkb/Q9UI32/entry" } ], "route": "amino-entry", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "Matrix glutamine-to-glutamate connection after the separately represented import event.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-15134", "name": "Glutamate dehydrogenase — NAD-dependent", "equation": "L-glutamate + NAD(+) + H2O =\u003e 2-oxoglutarate + NH4(+) + NADH + H(+)", "source_id": "rhea", "source_record_ids": [ "RHEA:15134" ], "source_url": "https://www.rhea-db.org/rhea/15134", "rhea_parent_id": "RHEA:15133", "human_support": [ { "source": "Reactome", "id": "R-HSA-70600", "url": "https://reactome.org/content/detail/R-HSA-70600" }, { "source": "UniProtKB", "id": "P00367", "url": "https://www.uniprot.org/uniprotkb/P00367/entry" }, { "source": "UniProtKB", "id": "P49448", "url": "https://www.uniprot.org/uniprotkb/P49448/entry" } ], "route": "amino-entry", "compartment": "mitochondrial matrix", "representation": "cofactor_specific_alternative", "visual_style": "solid", "direction_note": "NAD-dependent equation remains distinct from the NADP-dependent alternative.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-11613", "name": "Glutamate dehydrogenase — NADP-dependent", "equation": "L-glutamate + NADP(+) + H2O =\u003e 2-oxoglutarate + NH4(+) + NADPH + H(+)", "source_id": "rhea", "source_record_ids": [ "RHEA:11613" ], "source_url": "https://www.rhea-db.org/rhea/11613", "rhea_parent_id": "RHEA:11612", "human_support": [ { "source": "Reactome", "id": "R-HSA-70600", "url": "https://reactome.org/content/detail/R-HSA-70600" }, { "source": "UniProtKB", "id": "P00367", "url": "https://www.uniprot.org/uniprotkb/P00367/entry" }, { "source": "UniProtKB", "id": "P49448", "url": "https://www.uniprot.org/uniprotkb/P49448/entry" } ], "route": "amino-entry", "compartment": "mitochondrial matrix", "representation": "cofactor_specific_alternative", "visual_style": "solid", "direction_note": "NADP-dependent equation remains distinct from the NAD-dependent alternative.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-21825", "name": "Aspartate connection through GOT2", "equation": "L-aspartate + 2-oxoglutarate =\u003e oxaloacetate + L-glutamate", "source_id": "rhea", "source_record_ids": [ "RHEA:21825" ], "source_url": "https://www.rhea-db.org/rhea/21825", "rhea_parent_id": "RHEA:21824", "human_support": [ { "source": "Reactome", "id": "R-HSA-70596", "url": "https://reactome.org/content/detail/R-HSA-70596" }, { "source": "UniProtKB", "id": "P00505", "url": "https://www.uniprot.org/uniprotkb/P00505/entry" } ], "route": "amino-entry", "compartment": "mitochondrial matrix", "representation": "tca_pool_interconversion", "visual_style": "solid", "direction_note": "A TCA-pool interconversion, not standalone evidence of net anaplerosis.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-23721", "name": "Propionyl-CoA carboxylase", "equation": "propanoyl-CoA + hydrogencarbonate + ATP =\u003e (S)-methylmalonyl-CoA + ADP + phosphate + H(+)", "source_id": "rhea", "source_record_ids": [ "RHEA:23721" ], "source_url": "https://www.rhea-db.org/rhea/23721", "rhea_parent_id": "RHEA:23720", "human_support": [ { "source": "Reactome", "id": "R-HSA-71031", "url": "https://reactome.org/content/detail/R-HSA-71031" }, { "source": "UniProtKB", "id": "P05165", "url": "https://www.uniprot.org/uniprotkb/P05165/entry" }, { "source": "UniProtKB", "id": "P05166", "url": "https://www.uniprot.org/uniprotkb/P05166/entry" } ], "route": "propionyl-entry", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "Branch begins at propanoyl-CoA; no common upstream origin is implied.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-20555", "name": "Methylmalonyl-CoA epimerase", "equation": "(S)-methylmalonyl-CoA =\u003e (R)-methylmalonyl-CoA", "source_id": "rhea", "source_record_ids": [ "RHEA:20555" ], "source_url": "https://www.rhea-db.org/rhea/20555", "rhea_parent_id": "RHEA:20553", "human_support": [ { "source": "Reactome", "id": "R-HSA-71020", "url": "https://reactome.org/content/detail/R-HSA-71020" }, { "source": "UniProtKB", "id": "Q96PE7", "url": "https://www.uniprot.org/uniprotkb/Q96PE7/entry" } ], "route": "propionyl-entry", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "Stereochemical identities are preserved.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-22889", "name": "Methylmalonyl-CoA mutase", "equation": "(R)-methylmalonyl-CoA =\u003e succinyl-CoA", "source_id": "rhea", "source_record_ids": [ "RHEA:22889" ], "source_url": "https://www.rhea-db.org/rhea/22889", "rhea_parent_id": "RHEA:22888", "human_support": [ { "source": "Reactome", "id": "R-HSA-71010", "url": "https://reactome.org/content/detail/R-HSA-71010" }, { "source": "UniProtKB", "id": "P22033", "url": "https://www.uniprot.org/uniprotkb/P22033/entry" } ], "route": "propionyl-entry", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "Matrix connection to succinyl-CoA; no downstream flux is implied.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-10389", "name": "Mitochondrial phosphoenolpyruvate carboxykinase", "equation": "oxaloacetate + GTP =\u003e phosphoenolpyruvate + GDP + CO2", "source_id": "rhea", "source_record_ids": [ "RHEA:10389" ], "source_url": "https://www.rhea-db.org/rhea/10389", "rhea_parent_id": "RHEA:10388", "human_support": [ { "source": "Reactome", "id": "R-HSA-372819", "url": "https://reactome.org/content/detail/R-HSA-372819" }, { "source": "UniProtKB", "id": "Q16822", "url": "https://www.uniprot.org/uniprotkb/Q16822/entry" } ], "route": "cataplerosis", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "PCK2 matrix reaction. PEP transport is not inferred by this chemical edge.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "rhea-18254", "name": "Mitochondrial NADP-dependent malic enzyme", "equation": "(S)-malate + NADP(+) =\u003e pyruvate + CO2 + NADPH", "source_id": "rhea", "source_record_ids": [ "RHEA:18254" ], "source_url": "https://www.rhea-db.org/rhea/18254", "rhea_parent_id": "RHEA:18253", "human_support": [ { "source": "Reactome", "id": "R-HSA-9012349", "url": "https://reactome.org/content/detail/R-HSA-9012349" }, { "source": "UniProtKB", "id": "Q16798", "url": "https://www.uniprot.org/uniprotkb/Q16798/entry" } ], "route": "cataplerosis", "compartment": "mitochondrial matrix", "representation": "single_event", "visual_style": "solid", "direction_note": "ME3 matrix reaction. No cross-membrane movement or net tissue flux is inferred.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" }, { "id": "reactome-75849", "name": "Citrate / malate exchange", "equation": "", "source_id": "reactome", "source_record_ids": [ "R-HSA-75849" ], "source_url": "https://reactome.org/content/detail/R-HSA-75849", "rhea_parent_id": "", "human_support": [], "route": "transport", "compartment": "mitochondrial inner membrane", "representation": "transport_exchange", "visual_style": "dotted", "direction_note": "SLC25A1-mediated exchange, not passive citrate diffusion or a one-way chemical reaction.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": true, "transport_species": [ "citrate (mitochondrial matrix)", "citrate (cytosol)", "(S)-malate (cytosol)", "(S)-malate (mitochondrial matrix)" ], "qualifying_language": "exchange" }, { "id": "rhea-21162", "name": "ATP-citrate lyase", "equation": "citrate + ATP + CoA =\u003e oxaloacetate + acetyl-CoA + ADP + phosphate", "source_id": "rhea", "source_record_ids": [ "RHEA:21162" ], "source_url": "https://www.rhea-db.org/rhea/21162", "rhea_parent_id": "", "human_support": [ { "source": "Reactome", "id": "R-HSA-75848", "url": "https://reactome.org/content/detail/R-HSA-75848" }, { "source": "UniProtKB", "id": "P53396", "url": "https://www.uniprot.org/uniprotkb/P53396/entry" } ], "route": "cataplerosis", "compartment": "cytosol", "representation": "single_event", "visual_style": "solid", "direction_note": "Cytosolic cleavage after the separately represented citrate/malate exchange; no downstream lipid use is asserted.", "confidence_status": "audited-source-linked", "crosses_compartment_boundary": false, "transport_species": [], "qualifying_language": "" } ], "events": [ { "id": "R-HSA-109278", "label": "NT5E:Zn2+ hydrolyses AMP,dAMP,GMP, IMP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "extracellular region · plasma membrane", "compartment_ids": [ "GO:0005576", "GO:0005886" ], "rhea_ids": [ "RHEA:29376", "RHEA:29372", "RHEA:27715", "RHEA:27719" ], "source_url": "https://reactome.org/content/detail/R-HSA-109278", "summary": "NT5E:Zn2+ hydrolyses AMP,dAMP,GMP, IMP is a Reactome release 97 curated reaction event. The source record connects AMP, dAMP, GMP, IMP, H2O to Ade-Rib, dA, Gua-Rib, Ino, Pi in extracellular region · plasma membrane and cross-references RHEA:29376, RHEA:29372, RHEA:27715, RHEA:27719.", "equation": "AMP, dAMP, GMP, IMP + H2O → Ade-Rib, dA, Gua-Rib, Ino + Pi", "inputs": [ { "node_id": "R-ALL-500101", "reactome_id": "R-ALL-500101", "label": "AMP, dAMP, GMP, IMP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500104", "reactome_id": "R-ALL-500104", "label": "Ade-Rib, dA, Gua-Rib, Ino", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-109277", "reactome_id": "R-ALL-109277", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5'-nucleotidase activity of NT5E:Zn2+ dimer [plasma membrane]", "activity_id": "109273", "ec_numbers": [ "3.1.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:3036115", "title": "Purification of 5'-nucleotidase from human placenta after release from plasma membranes by phosphatidylinositol-specific phospholipase C", "journal": "Biochem Biophys Res Commun", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3036115/" }, { "id": "PMID:2129526", "title": "Primary structure of human placental 5'-nucleotidase and identification of the glycolipid anchor in the mature form", "journal": "Eur J Biochem", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2129526/" }, { "id": "PMID:1637327", "title": "5'-nucleotidase: molecular structure and functional aspects", "journal": "Biochem J", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1637327/" } ], "x": 2660, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-109380", "label": "(d)CMP, TMP, or (d)UMP + H2O =\u003e (deoxy)cytidine, thymidine, or (deoxy)uridine + orthophosphate (NT5C1A)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11081", "RHEA:29368", "RHEA:29364", "RHEA:29360", "RHEA:29356" ], "source_url": "https://reactome.org/content/detail/R-HSA-109380", "summary": "(d)CMP, TMP, or (d)UMP + H2O =\u003e (deoxy)cytidine, thymidine, or (deoxy)uridine + orthophosphate (NT5C1A) is a Reactome release 97 curated reaction event. The source record connects (d)CMP, TMP, (d)UMP, H2O to (d)C, T, (d)U, Pi in cytosol and cross-references RHEA:11081, RHEA:29368, RHEA:29364, RHEA:29360, RHEA:29356.", "equation": "(d)CMP, TMP, (d)UMP + H2O → (d)C, T, (d)U + Pi", "inputs": [ { "node_id": "R-ALL-500347", "reactome_id": "R-ALL-500347", "label": "(d)CMP, TMP, (d)UMP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500346", "reactome_id": "R-ALL-500346", "label": "(d)C, T, (d)U", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5'-nucleotidase activity of NT5C1A tetramer [cytosol]", "activity_id": "109365", "ec_numbers": [ "3.1.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:12947102", "title": "Mammalian 5'-nucleotidases", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12947102/" }, { "id": "PMID:11133996", "title": "Human cytosolic 5'-nucleotidase I. Characterization and role in nucleoside analog resistance", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11133996/" }, { "id": "PMID:10364222", "title": "The mechanism of adenosine formation in cells. Cloning of cytosolic 5'-nucleotidase-I", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10364222/" }, { "id": "PMID:2344353", "title": "Partial purification and properties of an AMP-specific soluble 5'-nucleotidase from pigeon heart", "journal": "Biochem J", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2344353/" } ], "x": 2381.3333333333335, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-109387", "label": "(2'-deoxy)purine nucleoside 5'-monophosphate + H2O =\u003e (2'-deoxy)purine nucleoside + orthophosphate [NT5C1A]", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:29376", "RHEA:29372", "RHEA:27715", "RHEA:29380", "RHEA:27719", "RHEA:29384" ], "source_url": "https://reactome.org/content/detail/R-HSA-109387", "summary": "(2'-deoxy)purine nucleoside 5'-monophosphate + H2O =\u003e (2'-deoxy)purine nucleoside + orthophosphate [NT5C1A] is a Reactome release 97 curated reaction event. The source record connects (d)AMP, (d)GMP, (d)IMP, H2O to (d)A, (d)G, (d)I, Pi in cytosol and cross-references RHEA:29376, RHEA:29372, RHEA:27715, RHEA:29380, RHEA:27719, RHEA:29384.", "equation": "(d)AMP, (d)GMP, (d)IMP + H2O → (d)A, (d)G, (d)I + Pi", "inputs": [ { "node_id": "R-ALL-500125", "reactome_id": "R-ALL-500125", "label": "(d)AMP, (d)GMP, (d)IMP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500124", "reactome_id": "R-ALL-500124", "label": "(d)A, (d)G, (d)I", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5'-nucleotidase activity of NT5C1A tetramer [cytosol]", "activity_id": "109365", "ec_numbers": [ "3.1.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:12947102", "title": "Mammalian 5'-nucleotidases", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12947102/" }, { "id": "PMID:11133996", "title": "Human cytosolic 5'-nucleotidase I. Characterization and role in nucleoside analog resistance", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11133996/" }, { "id": "PMID:10364222", "title": "The mechanism of adenosine formation in cells. Cloning of cytosolic 5'-nucleotidase-I", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10364222/" }, { "id": "PMID:2344353", "title": "Partial purification and properties of an AMP-specific soluble 5'-nucleotidase from pigeon heart", "journal": "Biochem J", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2344353/" } ], "x": 2660, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-109449", "label": "(d)CMP, TMP, or (d)UMP + H2O =\u003e (deoxy)cytidine, thymidine, or (deoxy)uridine + orthophosphate (NT5C3)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11081", "RHEA:29368", "RHEA:29364", "RHEA:29360", "RHEA:29356" ], "source_url": "https://reactome.org/content/detail/R-HSA-109449", "summary": "(d)CMP, TMP, or (d)UMP + H2O =\u003e (deoxy)cytidine, thymidine, or (deoxy)uridine + orthophosphate (NT5C3) is a Reactome release 97 curated reaction event. The source record connects (d)CMP, TMP, (d)UMP, H2O to (d)C, T, (d)U, Pi in cytosol and cross-references RHEA:11081, RHEA:29368, RHEA:29364, RHEA:29360, RHEA:29356.", "equation": "(d)CMP, TMP, (d)UMP + H2O → (d)C, T, (d)U + Pi", "inputs": [ { "node_id": "R-ALL-500347", "reactome_id": "R-ALL-500347", "label": "(d)CMP, TMP, (d)UMP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500346", "reactome_id": "R-ALL-500346", "label": "(d)C, T, (d)U", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5'-nucleotidase activity of NT5C3 holoenzyme [cytosol]", "activity_id": "109441", "ec_numbers": [ "3.1.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:11795870", "title": "Human erythrocyte pyrimidine 5'-nucleotidase, PN-I", "journal": "Arch Biochem Biophys", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11795870/" }, { "id": "PMID:9428647", "title": "Pyrimidine nucleotidases from human erythrocyte possess phosphotransferase activities specific for pyrimidine nucleotides", "journal": "FEBS Lett", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9428647/" }, { "id": "PMID:11369620", "title": "Genetic basis of hemolytic anemia caused by pyrimidine 5' nucleotidase deficiency", "journal": "Blood", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11369620/" }, { "id": "PMID:12580951", "title": "Pyrimidine 5' nucleotidase deficiency", "journal": "Br J Haematol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12580951/" } ], "x": 2938.666666666667, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-109470", "label": "(d)GMP or (d)IMP + H2O =\u003e (d)G or (d)I + orthophosphate (NT5C)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:27715", "RHEA:29380", "RHEA:27719", "RHEA:29384" ], "source_url": "https://reactome.org/content/detail/R-HSA-109470", "summary": "(d)GMP or (d)IMP + H2O =\u003e (d)G or (d)I + orthophosphate (NT5C) is a Reactome release 97 curated reaction event. The source record connects (d)GMP, (d)IMP, H2O to Gu-Rib, dG, Ino, dI, Pi in cytosol and cross-references RHEA:27715, RHEA:29380, RHEA:27719, RHEA:29384.", "equation": "(d)GMP, (d)IMP + H2O → Gu-Rib, dG, Ino, dI + Pi", "inputs": [ { "node_id": "R-ALL-500120", "reactome_id": "R-ALL-500120", "label": "(d)GMP, (d)IMP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500118", "reactome_id": "R-ALL-500118", "label": "Gu-Rib, dG, Ino, dI", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5'-nucleotidase activity of NT5C dimer [cytosol]", "activity_id": "109469", "ec_numbers": [ "3.1.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:2157703", "title": "Cytoplasmic 5'(3')-nucleotidase from human placenta", "journal": "J Biol Chem", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2157703/" }, { "id": "PMID:11083867", "title": "Cytosolic high Km 5'-nucleotidase and 5'(3')-deoxyribonucleotidase in substrate cycles involved in nucleotide metabolism", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11083867/" }, { "id": "PMID:10681516", "title": "Mammalian 5'(3')-deoxyribonucleotidase, cDNA cloning, and overexpression of the enzyme in Escherichia coli and mammalian cells", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10681516/" } ], "x": 2938.666666666667, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-109480", "label": "TMP, uridine 2', 3', or 5' monophosphates, or deoxyuridine 3' or 5' monophosphates + H2O =\u003e thymidine or (deoxy)uridine + orthophosphate [NT5C]", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11081", "RHEA:37356", "RHEA:27891", "RHEA:29360", "RHEA:29356" ], "source_url": "https://reactome.org/content/detail/R-HSA-109480", "summary": "TMP, uridine 2', 3', or 5' monophosphates, or deoxyuridine 3' or 5' monophosphates + H2O =\u003e thymidine or (deoxy)uridine + orthophosphate [NT5C] is a Reactome release 97 curated reaction event. The source record connects TMP, U2MP, U3MP, UMP, dU3MP, dUMP, H2O to Thy-Rib, (d)U, Pi in cytosol and cross-references RHEA:11081, RHEA:37356, RHEA:27891, RHEA:29360, RHEA:29356.", "equation": "TMP, U2MP, U3MP, UMP, dU3MP, dUMP + H2O → Thy-Rib, (d)U + Pi", "inputs": [ { "node_id": "R-ALL-500414", "reactome_id": "R-ALL-500414", "label": "TMP, U2MP, U3MP, UMP, dU3MP, dUMP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500415", "reactome_id": "R-ALL-500415", "label": "Thy-Rib, (d)U", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5'-nucleotidase activity of NT5C dimer [cytosol]", "activity_id": "109469", "ec_numbers": [ "3.1.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:11083867", "title": "Cytosolic high Km 5'-nucleotidase and 5'(3')-deoxyribonucleotidase in substrate cycles involved in nucleotide metabolism", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11083867/" }, { "id": "PMID:2157703", "title": "Cytoplasmic 5'(3')-nucleotidase from human placenta", "journal": "J Biol Chem", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2157703/" }, { "id": "PMID:10681516", "title": "Mammalian 5'(3')-deoxyribonucleotidase, cDNA cloning, and overexpression of the enzyme in Escherichia coli and mammalian cells", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10681516/" } ], "x": 2381.3333333333335, "y": 3622.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-109514", "label": "TMP, (d)UMP, uridine 2' monophosphate, or uridine 3'-monophosphate + H2O =\u003e thymidine, deoxyuridine, or uridine + orthophosphate [NT5M]", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:11081", "RHEA:29360", "RHEA:29356", "RHEA:37356", "RHEA:27891" ], "source_url": "https://reactome.org/content/detail/R-HSA-109514", "summary": "TMP, (d)UMP, uridine 2' monophosphate, or uridine 3'-monophosphate + H2O =\u003e thymidine, deoxyuridine, or uridine + orthophosphate [NT5M] is a Reactome release 97 curated reaction event. The source record connects TMP, UMP, dU5MP, U2MP, U3MP, H2O to Thy-dRib, Ura-Rib, dU, Pi in mitochondrial matrix and cross-references RHEA:11081, RHEA:29360, RHEA:29356, RHEA:37356, RHEA:27891.", "equation": "TMP, UMP, dU5MP, U2MP, U3MP + H2O → Thy-dRib, Ura-Rib, dU + Pi", "inputs": [ { "node_id": "R-ALL-500420", "reactome_id": "R-ALL-500420", "label": "TMP, UMP, dU5MP, U2MP, U3MP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500421", "reactome_id": "R-ALL-500421", "label": "Thy-dRib, Ura-Rib, dU", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5'-nucleotidase activity of NT5M dimer [mitochondrial matrix]", "activity_id": "109498", "ec_numbers": [ "3.1.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:10899995", "title": "A deoxynucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease", "journal": "Proc Natl Acad Sci USA", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10899995/" }, { "id": "PMID:12124385", "title": "Human mitochondrial 5'-deoxynucleotidase. Overproduction in cultured cells and functional aspects", "journal": "J Biol Chem", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12124385/" }, { "id": "PMID:12352955", "title": "Crystal structure of a human mitochondrial deoxyribonuclease", "journal": "Nt Struct Biol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12352955/" } ], "x": 2660, "y": 3622.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-109759", "label": "deoxycytidine, thymidine, or deoxyuridine + ATP =\u003e dCMP, TMP, or dUMP + ADP [TK2]", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:46041", "RHEA:19130", "RHEA:28207" ], "source_url": "https://reactome.org/content/detail/R-HSA-109759", "summary": "deoxycytidine, thymidine, or deoxyuridine + ATP =\u003e dCMP, TMP, or dUMP + ADP [TK2] is a Reactome release 97 curated reaction event. The source record connects dC, Thy-dRib, dU, ATP to dCMP, TMP, dUMP, ADP, H+ in mitochondrial matrix and cross-references RHEA:46041, RHEA:19130, RHEA:28207.", "equation": "dC, Thy-dRib, dU + ATP → dCMP, TMP, dUMP + ADP + H+", "inputs": [ { "node_id": "R-ALL-500822", "reactome_id": "R-ALL-500822", "label": "dC, Thy-dRib, dU", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500823", "reactome_id": "R-ALL-500823", "label": "dCMP, TMP, dUMP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside kinase activity of TK2 [mitochondrial matrix]", "activity_id": "109753", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:1597187", "title": "Mammalian thymidine kinase 2. Direct photoaffinity labeling with [32P]dTTP of the enzyme from spleen, liver, heart and brain", "journal": "Eur J Biochem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1597187/" }, { "id": "PMID:1063125", "title": "Human deoxythymidine kinase. I. Purification and general properties of the cytoplasmic and mitochondrial isozymes derived from blast cells of acute myelocytic leukemia", "journal": "J Biol Chem", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/1063125/" }, { "id": "PMID:2026611", "title": "Diverging substrate specificity of pure human thymidine kinases 1 and 2 against antiviral dideoxynucleosides", "journal": "J Biol Chem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/2026611/" }, { "id": "PMID:9989599", "title": "Human thymidine kinase 2: molecular cloning and characterisation of the enzyme activity with antiviral and cytostatic nucleoside substrates", "journal": "FEBS Lett", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/9989599/" } ], "x": 3600, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-110133", "label": "(d)GDP + ADP (d)GMP + ATP (GUK1)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:12699", "RHEA:20782" ], "source_url": "https://reactome.org/content/detail/R-HSA-110133", "summary": "(d)GDP + ADP (d)GMP + ATP (GUK1) is a Reactome release 97 curated reaction event. The source record connects (d)GDP, ADP to (d)GMP, ATP in cytosol and cross-references RHEA:12699, RHEA:20782.", "equation": "(d)GDP + ADP → (d)GMP + ATP", "inputs": [ { "node_id": "R-ALL-500077", "reactome_id": "R-ALL-500077", "label": "(d)GDP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500071", "reactome_id": "R-ALL-500071", "label": "(d)GMP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "GMP kinase activity of GUK1 [cytosol]", "activity_id": "74189", "ec_numbers": [ "2.7.4.8" ], "accessions": null } ], "citations": [ { "id": "PMID:211390", "title": "Guanylate kinases from human erythrocytes, hog brain, and rat liver", "journal": "Methods Enzymol", "year": 1978, "url": "https://pubmed.ncbi.nlm.nih.gov/211390/" }, { "id": "PMID:8663313", "title": "Cloning, characterization, and modeling of mouse and human guanylate kinases", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8663313/" }, { "id": "PMID:177353", "title": "Studies on the properties and tissue distribution of the isozymes of guanylate kinase in man", "journal": "Human Hered", "year": 1975, "url": "https://pubmed.ncbi.nlm.nih.gov/177353/" }, { "id": "PMID:6248551", "title": "Phosphorylation of acyclovir (acycloguanosine) monophosphate by GMP kinase", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6248551/" } ], "x": 3321.3333333333335, "y": 3622.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-110137", "label": "(d)ADP or (d)CDP + ADP (d)AMP or (d)CMP + ATP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23102", "RHEA:12975", "RHEA:11602", "RHEA:25096" ], "source_url": "https://reactome.org/content/detail/R-HSA-110137", "summary": "(d)ADP or (d)CDP + ADP (d)AMP or (d)CMP + ATP is a Reactome release 97 curated reaction event. The source record connects (d)NDPs, ADP to (d)NMPs, ATP in cytosol and cross-references RHEA:23102, RHEA:12975, RHEA:11602, RHEA:25096.", "equation": "(d)NDPs + ADP → (d)NMPs + ATP", "inputs": [ { "node_id": "R-ALL-6788782", "reactome_id": "R-ALL-6788782", "label": "(d)NDPs", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6788778", "reactome_id": "R-ALL-6788778", "label": "(d)NMPs", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside kinase activity of AK5,7,8,9 [cytosol]", "activity_id": "110088", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10215863", "title": "Identification of a novel human adenylate kinase. cDNA cloning, expression analysis, chromosome localization and characterization of the recombinant protein", "journal": "Eur J Biochem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10215863/" } ], "x": 3321.3333333333335, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-110138", "label": "AK5,7,8,9 phosphorylates (d)NMPs to (d)NDPs", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23101", "RHEA:12974", "RHEA:11601", "RHEA:25095" ], "source_url": "https://reactome.org/content/detail/R-HSA-110138", "summary": "AK5,7,8,9 phosphorylates (d)NMPs to (d)NDPs is a Reactome release 97 curated reaction event. The source record connects (d)NMPs, ATP to (d)NDPs, ADP in cytosol and cross-references RHEA:23101, RHEA:12974, RHEA:11601, RHEA:25095.", "equation": "(d)NMPs + ATP → (d)NDPs + ADP", "inputs": [ { "node_id": "R-ALL-6788778", "reactome_id": "R-ALL-6788778", "label": "(d)NMPs", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6788782", "reactome_id": "R-ALL-6788782", "label": "(d)NDPs", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside kinase activity of AK5,7,8,9 [cytosol]", "activity_id": "110088", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10215863", "title": "Identification of a novel human adenylate kinase. cDNA cloning, expression analysis, chromosome localization and characterization of the recombinant protein", "journal": "Eur J Biochem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10215863/" }, { "id": "PMID:21080915", "title": "The characterization of human adenylate kinases 7 and 8 demonstrates differences in kinetic parameters and structural organization among the family of adenylate kinase isoenzymes", "journal": "Biochem. J.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21080915/" }, { "id": "PMID:23416111", "title": "The human adenylate kinase 9 is a nucleoside mono- and diphosphate kinase", "journal": "Int. J. Biochem. Cell Biol.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23416111/" } ], "x": 3600, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-111524", "label": "IMP + L-Aspartate + GTP =\u003e Adenylosuccinate + GDP + Pi [ADSS]", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15754" ], "source_url": "https://reactome.org/content/detail/R-HSA-111524", "summary": "IMP + L-Aspartate + GTP =\u003e Adenylosuccinate + GDP + Pi [ADSS] is a Reactome release 97 curated reaction event. The source record connects GTP, IMP, L-Asp to GDP, Pi, ADS in cytosol and cross-references RHEA:15754.", "equation": "GTP + IMP + L-Asp → GDP + Pi + ADS + 2 H+", "inputs": [ { "node_id": "R-ALL-29438", "reactome_id": "R-ALL-29438", "label": "GTP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:37565", "formula": "C10H12N5O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29602", "reactome_id": "R-ALL-29602", "label": "IMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58053", "formula": "C10H11N4O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29448", "reactome_id": "R-ALL-29448", "label": "L-Asp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29420", "reactome_id": "R-ALL-29420", "label": "GDP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58189", "formula": "C10H12N5O11P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-35141", "reactome_id": "R-ALL-35141", "label": "ADS", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57567", "formula": "C14H14N5O11P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "adenylosuccinate synthase activity of ADSS, ADSSL1 dimers [cytosol]", "activity_id": "111492", "ec_numbers": [ "6.3.4.4" ], "accessions": null } ], "citations": [ { "id": "PMID:1592113", "title": "Cloning and characterization of the cDNA encoding human adenylosuccinate synthetase", "journal": "FEBS Lett", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1592113/" }, { "id": "PMID:15786719", "title": "Molecular cloning and characterization of a novel muscle adenylosuccinate synthetase, AdSSL1, from human bone marrow stromal cells", "journal": "Mol Cell Biochem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15786719/" } ], "x": 2381.3333333333335, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-111883", "label": "PLA2G4A (cPLA2) hydrolyzes phosphatidylcholine", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "endoplasmic reticulum membrane · cytosol · endoplasmic reticulum lumen", "compartment_ids": [ "GO:0005789", "GO:0005829", "GO:0005788" ], "rhea_ids": [ "RHEA:40652", "RHEA:40651" ], "source_url": "https://reactome.org/content/detail/R-HSA-111883", "summary": "PLA2G4A (cPLA2) hydrolyzes phosphatidylcholine is a Reactome release 97 curated reaction event. The source record connects H2O, AA-PC to AA, H+, LysoPtdCho in endoplasmic reticulum membrane · cytosol · endoplasmic reticulum lumen and cross-references RHEA:40652, RHEA:40651.", "equation": "H2O + AA-PC → AA + H+ + LysoPtdCho", "inputs": [ { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9915525", "reactome_id": "R-ALL-9915525", "label": "AA-PC", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:75063", "formula": "C29H49NO8PR", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29768", "reactome_id": "R-ALL-29768", "label": "AA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:32395", "formula": "C20H31O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-6814253", "reactome_id": "R-ALL-6814253", "label": "LysoPtdCho", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58168", "formula": "C9H19NO7PR", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "A2-type glycerophospholipase activity of Active PLA2:phosphatidylcholine [endoplasmic reticulum membrane]", "activity_id": "111882", "ec_numbers": [ "3.1.1.4" ], "accessions": null } ], "citations": [ { "id": "PMID:21247147", "title": "Functional characterization of mutations in inherited human cPLA? deficiency", "journal": "Biochemistry", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21247147/" }, { "id": "PMID:21746768", "title": "Secreted phospholipase A2 revisited", "journal": "J. Biochem.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21746768/" } ], "x": 4451.333333333333, "y": 2076.8333333333335, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-112033", "label": "PNP catalyzes the conversion of hypoxanthine and (deoxy)ribose-1-phosphate to (deoxy)inosine", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:27648", "RHEA:27752" ], "source_url": "https://reactome.org/content/detail/R-HSA-112033", "summary": "PNP catalyzes the conversion of hypoxanthine and (deoxy)ribose-1-phosphate to (deoxy)inosine is a Reactome release 97 curated reaction event. The source record connects Hyp, R1P, dRibP to Pi, Ino, dI in cytosol and cross-references RHEA:27648, RHEA:27752.", "equation": "Hyp + R1P, dRibP → Pi + Ino, dI", "inputs": [ { "node_id": "R-ALL-113599", "reactome_id": "R-ALL-113599", "label": "Hyp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17368", "formula": "C5H4N4O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-500244", "reactome_id": "R-ALL-500244", "label": "R1P, dRibP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-500172", "reactome_id": "R-ALL-500172", "label": "Ino, dI", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "purine-nucleoside phosphorylase activity of PNP trimer [cytosol]", "activity_id": "74238", "ec_numbers": [ "2.4.2.1" ], "accessions": null } ], "citations": [ { "id": "PMID:6771276", "title": "Characterization of purine nucleoside phosphorylase from human granulocytes and its metabolism of deoxyribonucleosides", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6771276/" }, { "id": "PMID:3029074", "title": "A human purine nucleoside phosphorylase deficiency caused by a single base change", "journal": "J Biol Chem", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3029074/" }, { "id": "PMID:2104852", "title": "Three-dimensional structure of human erythrocytic purine nucleoside phosphorylase at 3.2 A resolution", "journal": "J Biol Chem", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2104852/" } ], "x": 3878.666666666667, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1237045", "label": "Carbonic Anhydrase VI hydrates carbon dioxide to bicarbonate and a proton", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "extracellular region", "compartment_ids": [ "GO:0005576" ], "rhea_ids": [ "RHEA:10750" ], "source_url": "https://reactome.org/content/detail/R-HSA-1237045", "summary": "Carbonic Anhydrase VI hydrates carbon dioxide to bicarbonate and a proton is a Reactome release 97 curated reaction event. The source record connects CO2, H2O to H+, HCO3- in extracellular region and cross-references RHEA:10750.", "equation": "CO2 + H2O → H+ + HCO3-", "inputs": [ { "node_id": "R-ALL-1237009", "reactome_id": "R-ALL-1237009", "label": "CO2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-425425", "reactome_id": "R-ALL-425425", "label": "HCO3-", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA6:Zinc [extracellular region]", "activity_id": "1237060", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:9784398", "title": "Gustin from human parotid saliva is carbonic anhydrase VI", "journal": "Biochem Biophys Res Commun", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9784398/" }, { "id": "PMID:17499996", "title": "Carbonic anhydrase activators: the first activation study of the human secretory isoform VI with amino acids and amines", "journal": "Bioorg Med Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17499996/" }, { "id": "PMID:17228881", "title": "Carbonic anhydrase inhibitors. DNA cloning, characterization, and inhibition studies of the human secretory isoform VI, a new target for sulfonamide and sulfamate inhibitors", "journal": "J Med Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17228881/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" } ], "x": 6521.333333333333, "y": 806.8333333333334, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1237081", "label": "Carbonic anhydrase VI dehydrates bicarbonate to water and carbon dioxide", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "extracellular region", "compartment_ids": [ "GO:0005576" ], "rhea_ids": [ "RHEA:10749" ], "source_url": "https://reactome.org/content/detail/R-HSA-1237081", "summary": "Carbonic anhydrase VI dehydrates bicarbonate to water and carbon dioxide is a Reactome release 97 curated reaction event. The source record connects H+, HCO3- to CO2, H2O in extracellular region and cross-references RHEA:10749.", "equation": "H+ + HCO3- → CO2 + H2O", "inputs": [ { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-425425", "reactome_id": "R-ALL-425425", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1237009", "reactome_id": "R-ALL-1237009", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA6:Zinc [extracellular region]", "activity_id": "1237060", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:9784398", "title": "Gustin from human parotid saliva is carbonic anhydrase VI", "journal": "Biochem Biophys Res Commun", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9784398/" }, { "id": "PMID:17499996", "title": "Carbonic anhydrase activators: the first activation study of the human secretory isoform VI with amino acids and amines", "journal": "Bioorg Med Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17499996/" }, { "id": "PMID:17228881", "title": "Carbonic anhydrase inhibitors. DNA cloning, characterization, and inhibition studies of the human secretory isoform VI, a new target for sulfonamide and sulfamate inhibitors", "journal": "J Med Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17228881/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" } ], "x": 6800, "y": 806.8333333333334, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1237102", "label": "Transamination of MOB to methionine", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:31765" ], "source_url": "https://reactome.org/content/detail/R-HSA-1237102", "summary": "Transamination of MOB to methionine is a Reactome release 97 curated reaction event. The source record connects 4MTOBUTA, L-amino acid to L-Met, 2-Oxoacid in cytosol and cross-references RHEA:31765.", "equation": "4MTOBUTA + L-amino acid → L-Met + 2-Oxoacid", "inputs": [ { "node_id": "R-ALL-1237122", "reactome_id": "R-ALL-1237122", "label": "4MTOBUTA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16723", "formula": "C5H7O3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8942986", "reactome_id": "R-ALL-8942986", "label": "L-amino acid", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59869", "formula": "C2H4NO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-174390", "reactome_id": "R-ALL-174390", "label": "L-Met", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57844", "formula": "C5H11NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1237175", "reactome_id": "R-ALL-1237175", "label": "2-Oxoacid", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:35179", "formula": "C2O3R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "transaminase activity of PXLP-K259-GOT1 dimer [cytosol]", "activity_id": "1295495", "ec_numbers": [ "2.6.1" ], "accessions": null } ], "citations": [ { "id": "PMID:11443076", "title": "Methionine regeneration and aspartate aminotransferase in parasitic protozoa", "journal": "J Bacteriol", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11443076/" } ], "x": 1808.6666666666667, "y": 2994.1111111111113, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1237119", "label": "Acireductone is oxidized to MOB", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24505" ], "source_url": "https://reactome.org/content/detail/R-HSA-1237119", "summary": "Acireductone is oxidized to MOB is a Reactome release 97 curated reaction event. The source record connects Acireductone, O2 to 4MTOBUTA, HCOOH, H+ in cytosol and cross-references RHEA:24505.", "equation": "Acireductone + O2 → 4MTOBUTA + HCOOH + 2 H+", "inputs": [ { "node_id": "R-ALL-1237155", "reactome_id": "R-ALL-1237155", "label": "Acireductone", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:49252", "formula": "C6H10O3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1237122", "reactome_id": "R-ALL-1237122", "label": "4MTOBUTA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16723", "formula": "C5H7O3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29460", "reactome_id": "R-ALL-29460", "label": "HCOOH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15740", "formula": "CHO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acireductone dioxygenase [iron(II)-requiring] activity of ARD:Fe++ [cytosol]", "activity_id": "1237139", "ec_numbers": [ "1.13.11.54" ], "accessions": null } ], "citations": [ { "id": "PMID:17786183", "title": "Expression and function of the human androgen-responsive gene ADI1 in prostate cancer", "journal": "Neoplasia", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17786183/" } ], "x": 1530, "y": 2917.6666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1247910", "label": "CNDP2:2Mn2+ dimer hydrolyses CysGly", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:28784" ], "source_url": "https://reactome.org/content/detail/R-HSA-1247910", "summary": "CNDP2:2Mn2+ dimer hydrolyses CysGly is a Reactome release 97 curated reaction event. The source record connects CysGly, H2O to L-Cys, Gly in cytosol and cross-references RHEA:28784.", "equation": "CysGly + H2O → L-Cys + Gly", "inputs": [ { "node_id": "R-ALL-1247904", "reactome_id": "R-ALL-1247904", "label": "CysGly", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:61694", "formula": "C5H10N2O3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111707", "reactome_id": "R-ALL-111707", "label": "L-Cys", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:35235", "formula": "C3H7NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29424", "reactome_id": "R-ALL-29424", "label": "Gly", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carboxypeptidase activity of CNDP2:2Mn2+ dimer [cytosol]", "activity_id": "1258416", "ec_numbers": [ "3.4.17.11" ], "accessions": null } ], "citations": [ { "id": "PMID:12473676", "title": "Sequence identification and characterization of human carnosinase and a closely related non-specific dipeptidase", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12473676/" } ], "x": 311.33333333333337, "y": 3556.333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1247915", "label": "GSH translocates from cytosol to extracellular region", "schema_class": "BlackBoxEvent", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:74819" ], "source_url": "https://reactome.org/content/detail/R-HSA-1247915", "summary": "GSH translocates from cytosol to extracellular region is a Reactome release 97 curated black-box event whose internal chemistry is not expanded by the source record. The source record connects GSH to GSH in plasma membrane and cross-references RHEA:74819.", "equation": "GSH → GSH", "inputs": [ { "node_id": "R-ALL-29450", "reactome_id": "R-ALL-29450", "label": "GSH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57925", "formula": "C10H16N3O6S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1247892", "reactome_id": "R-ALL-1247892", "label": "GSH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16856", "formula": "C10H17N3O6S", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [], "citations": [ { "id": "PMID:34150", "title": "Translocation of intracellular glutathione to membrane-bound gamma-glutamyl transpeptidase as a discrete step in the gamma-glutamyl cycle: glutathionuria after inhibition of transpeptidase", "journal": "Proc Natl Acad Sci U S A", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/34150/" }, { "id": "PMID:42913", "title": "Transport of gamma-glutamyl amino acids: role of glutathione and gamma-glutamyl transpeptidase", "journal": "Proc Natl Acad Sci U S A", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/42913/" } ], "x": 311.33333333333337, "y": 3632.777777777778, "lod": "pathway", "collapsed": true, "transport": true }, { "id": "R-HSA-1247922", "label": "GGCT transforms gGluCys to OPRO", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-1247922", "summary": "GGCT transforms gGluCys to OPRO is a Reactome release 97 curated reaction event. The source record connects gGluCys to L-Cys, OPRO in cytosol.", "equation": "gGluCys → L-Cys + OPRO", "inputs": [ { "node_id": "R-ALL-174393", "reactome_id": "R-ALL-174393", "label": "gGluCys", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58173", "formula": "C8H13N2O5S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-111707", "reactome_id": "R-ALL-111707", "label": "L-Cys", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:35235", "formula": "C3H7NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1247942", "reactome_id": "R-ALL-1247942", "label": "OPRO", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58402", "formula": "C5H6NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "gamma-glutamylcyclotransferase activity of GGCT dimer [cytosol]", "activity_id": "1247932", "ec_numbers": [ "4.3.2.9" ], "accessions": null } ], "citations": [ { "id": "PMID:18515354", "title": "The identification and structural characterization of C7orf24 as gamma-glutamyl cyclotransferase. An essential enzyme in the gamma-glutamyl cycle", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18515354/" } ], "x": 590, "y": 3632.777777777778, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1247935", "label": "OPLAH hydrolyses OPRO to L-Glu", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10349" ], "source_url": "https://reactome.org/content/detail/R-HSA-1247935", "summary": "OPLAH hydrolyses OPRO to L-Glu is a Reactome release 97 curated reaction event. The source record connects ATP, H2O, OPRO to ADP, L-Glu, Pi in cytosol and cross-references RHEA:10349.", "equation": "ATP + 2 H2O + OPRO → ADP + L-Glu + Pi", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-1247942", "reactome_id": "R-ALL-1247942", "label": "OPRO", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58402", "formula": "C5H6NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5-oxoprolinase (ATP-hydrolyzing) activity of OPLAH [cytosol]", "activity_id": "1247941", "ec_numbers": [ "3.5.2.9" ], "accessions": null } ], "citations": [ { "id": "PMID:9516961", "title": "Characterization of 5-oxo-L-prolinase in normal and tumor tissues of humans and rats: a potential new target for biochemical modulation of glutathione", "journal": "Clin Cancer Res", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9516961/" } ], "x": 311.33333333333337, "y": 3479.8888888888887, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1362408", "label": "FXN:NFS1:ISD11:ISCU assembles 2Fe-2S iron-sulfur cluster", "schema_class": "BlackBoxEvent", "domain_id": "vitamins-cofactors", "pathway_ids": [ "iron-sulfur" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-1362408", "summary": "FXN:NFS1:ISD11:ISCU assembles 2Fe-2S iron-sulfur cluster is a Reactome release 97 curated black-box event whose internal chemistry is not expanded by the source record. The source record connects 2 Iron:FXN:NFS1:ISD11:ISCU, L-Cys, FDX1,FDX2 (reduced) to FXN:NFS1:ISD11:ISCU:2Fe-2S Cluster, L-Ala, FDX1,FDX2 (oxidized) in mitochondrial matrix.", "equation": "2 Iron:FXN:NFS1:ISD11:ISCU + 4 L-Cys + 4 FDX1,FDX2 (reduced) → FXN:NFS1:ISD11:ISCU:2Fe-2S Cluster + 4 L-Ala + 4 FDX1,FDX2 (oxidized)", "inputs": [ { "node_id": "R-HSA-1362401", "reactome_id": "R-HSA-1362401", "label": "2 Iron:FXN:NFS1:ISD11:ISCU", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-379721", "reactome_id": "R-ALL-379721", "label": "L-Cys", "role": "input", "stoichiometry": 4, "chebi_id": "CHEBI:35235", "formula": "C3H7NO2S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-2408372", "reactome_id": "R-HSA-2408372", "label": "FDX1,FDX2 (reduced)", "role": "input", "stoichiometry": 4, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-HSA-1362398", "reactome_id": "R-HSA-1362398", "label": "FXN:NFS1:ISD11:ISCU:2Fe-2S Cluster", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-379697", "reactome_id": "R-ALL-379697", "label": "L-Ala", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:57972", "formula": "C3H7NO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-2408370", "reactome_id": "R-HSA-2408370", "label": "FDX1,FDX2 (oxidized)", "role": "output", "stoichiometry": 4, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "cysteine desulfurase activity of 2 Iron:FXN:NFS1:ISD11:ISCU [mitochondrial matrix]", "activity_id": "1362414", "ec_numbers": [ "2.8.1.7" ], "accessions": null } ], "citations": [ { "id": "PMID:20873749", "title": "Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex", "journal": "Biochemistry", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20873749/" }, { "id": "PMID:19454487", "title": "Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis", "journal": "Hum Mol Genet", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19454487/" }, { "id": "PMID:17331979", "title": "Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones", "journal": "Hum Mol Genet", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17331979/" }, { "id": "PMID:12785837", "title": "Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins", "journal": "J Am Chem Soc", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12785837/" }, { "id": "PMID:22352884", "title": "Effector role reversal during evolution: the case of frataxin in Fe-S cluster biosynthesis", "journal": "Biochemistry", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22352884/" }, { "id": "PMID:22101253", "title": "Both human ferredoxins 1 and 2 and ferredoxin reductase are important for iron-sulfur cluster biogenesis", "journal": "Biochim. Biophys. Acta", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22101253/" } ], "x": 5948.666666666667, "y": 3573.75, "lod": "pathway", "collapsed": true, "transport": false }, { "id": "R-HSA-1362416", "label": "Frataxin binds iron", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "iron-sulfur" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-1362416", "summary": "Frataxin binds iron is a Reactome release 97 curated reaction event. The source record connects FXN:ISD11:NFS1:ISCU, Fe2+ to 2 Iron:FXN:NFS1:ISD11:ISCU in mitochondrial matrix.", "equation": "FXN:ISD11:NFS1:ISCU + 4 Fe2+ → 2 Iron:FXN:NFS1:ISD11:ISCU", "inputs": [ { "node_id": "R-HSA-1362404", "reactome_id": "R-HSA-1362404", "label": "FXN:ISD11:NFS1:ISCU", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113531", "reactome_id": "R-ALL-113531", "label": "Fe2+", "role": "input", "stoichiometry": 4, "chebi_id": "CHEBI:29033", "formula": "Fe", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-HSA-1362401", "reactome_id": "R-HSA-1362401", "label": "2 Iron:FXN:NFS1:ISD11:ISCU", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false } ], "catalysts": [ { "label": "ferrous iron binding activity of FXN:ISD11:NFS1:ISCU [mitochondrial matrix]", "activity_id": "9954151", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:20873749", "title": "Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex", "journal": "Biochemistry", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20873749/" }, { "id": "PMID:21298097", "title": "Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex", "journal": "PLoS One", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21298097/" }, { "id": "PMID:12785837", "title": "Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins", "journal": "J Am Chem Soc", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12785837/" }, { "id": "PMID:18425540", "title": "Mapping iron binding sites on human frataxin: implications for cluster assembly on the ISU Fe-S cluster scaffold protein", "journal": "J Biol Inorg Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18425540/" }, { "id": "PMID:11823441", "title": "Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia", "journal": "Hum Mol Genet", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11823441/" }, { "id": "PMID:21776984", "title": "Structure-function analysis of Friedreich's ataxia mutants reveals determinants of frataxin binding and activation of the Fe-S assembly complex", "journal": "Biochemistry", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21776984/" } ], "x": 5948.666666666667, "y": 3379.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1362417", "label": "Mitoferrin translocates iron from the mitochondrial intermembrane space to the mitochondrial matrix", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "iron-sulfur" ], "compartment": "mitochondrial inner membrane · mitochondrial intermembrane space · mitochondrial matrix", "compartment_ids": [ "GO:0005743", "GO:0005758", "GO:0005759" ], "rhea_ids": [ "RHEA:28486" ], "source_url": "https://reactome.org/content/detail/R-HSA-1362417", "summary": "Mitoferrin translocates iron from the mitochondrial intermembrane space to the mitochondrial matrix is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects Fe2+ to Fe2+ in mitochondrial inner membrane · mitochondrial intermembrane space · mitochondrial matrix and cross-references RHEA:28486.", "equation": "Fe2+ → Fe2+", "inputs": [ { "node_id": "R-ALL-1362385", "reactome_id": "R-ALL-1362385", "label": "Fe2+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29033", "formula": "Fe", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113531", "reactome_id": "R-ALL-113531", "label": "Fe2+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29033", "formula": "Fe", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "ferrous iron transmembrane transporter activity of Mitoferrin1,2 [mitochondrial inner membrane]", "activity_id": "1362410", "ec_numbers": null, "accessions": null } ], "citations": [], "x": 5391.333333333333, "y": 3379.25, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-140355", "label": "Arachidonate is oxidised to PGG2 by PTGS1", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "arachidonate", "biological-oxidations", "fatty-acid-metabolism", "phase-one" ], "compartment": "endoplasmic reticulum lumen · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005788", "GO:0005789" ], "rhea_ids": [ "RHEA:42597" ], "source_url": "https://reactome.org/content/detail/R-HSA-140355", "summary": "Arachidonate is oxidised to PGG2 by PTGS1 is a Reactome release 97 curated reaction event. The source record connects O2, AA to PGG2 in endoplasmic reticulum lumen · endoplasmic reticulum membrane and cross-references RHEA:42597.", "equation": "2 O2 + AA → PGG2", "inputs": [ { "node_id": "R-ALL-113534", "reactome_id": "R-ALL-113534", "label": "O2", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-140356", "reactome_id": "R-ALL-140356", "label": "AA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:32395", "formula": "C20H31O2", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-140357", "reactome_id": "R-ALL-140357", "label": "PGG2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:82629", "formula": "C20H31O6", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "prostaglandin-endoperoxide synthase activity of PTGS1 dimer [endoplasmic reticulum membrane]", "activity_id": "140354", "ec_numbers": [ "1.14.99.1" ], "accessions": null } ], "citations": [ { "id": "PMID:4521806", "title": "Isolation and structure of two prostaglandin endoperoxides that cause platelet aggregation", "journal": "Proc Natl Acad Sci U S A", "year": 1974, "url": "https://pubmed.ncbi.nlm.nih.gov/4521806/" }, { "id": "PMID:4776443", "title": "Isolation and properties of intermediates in prostaglandin biosynthesis", "journal": "Biochim Biophys Acta", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4776443/" } ], "x": 6521.333333333333, "y": 3379.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-140359", "label": "PGG2 is reduced to PGH2 by PTGS1", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "biological-oxidations", "fatty-acid-metabolism", "phase-one" ], "compartment": "endoplasmic reticulum lumen · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005788", "GO:0005789" ], "rhea_ids": [ "RHEA:42601", "RHEA:42600" ], "source_url": "https://reactome.org/content/detail/R-HSA-140359", "summary": "PGG2 is reduced to PGH2 by PTGS1 is a Reactome release 97 curated reaction event. The source record connects PGG2, hydrogen donor to H2O, PGH2, hydrogen acceptor in endoplasmic reticulum lumen · endoplasmic reticulum membrane and cross-references RHEA:42601, RHEA:42600.", "equation": "PGG2 + hydrogen donor → H2O + PGH2 + hydrogen acceptor", "inputs": [ { "node_id": "R-ALL-140357", "reactome_id": "R-ALL-140357", "label": "PGG2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:82629", "formula": "C20H31O6", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9915557", "reactome_id": "R-ALL-9915557", "label": "hydrogen donor", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17499", "formula": "H2A", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113519", "reactome_id": "R-ALL-113519", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-30138", "reactome_id": "R-ALL-30138", "label": "PGH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57405", "formula": "C20H31O5", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9915551", "reactome_id": "R-ALL-9915551", "label": "hydrogen acceptor", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13193", "formula": "A", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "peroxidase activity of PTGS1 dimer [endoplasmic reticulum membrane]", "activity_id": "140358", "ec_numbers": [ "1.11.1.7" ], "accessions": null } ], "citations": [ { "id": "PMID:4514999", "title": "Detection and isolation of an endoperoxide intermediate in prostaglandin biosynthesis", "journal": "Proc Natl Acad Sci U S A", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4514999/" }, { "id": "PMID:1380156", "title": "Human cyclooxygenase-2 cDNA", "journal": "Proc Natl Acad Sci U S A", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1380156/" }, { "id": "PMID:9139685", "title": "Differential allosteric regulation of prostaglandin H synthase 1 and 2 by arachidonic acid", "journal": "J Biol Chem", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9139685/" }, { "id": "PMID:7947975", "title": "Purification, characterization and selective inhibition of human prostaglandin G/H synthase 1 and 2 expressed in the baculovirus system", "journal": "Biochim Biophys Acta", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7947975/" } ], "x": 4730, "y": 2076.8333333333335, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-141341", "label": "SMOX-3 oxidises SPN to SPM", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-one" ], "compartment": "peroxisomal matrix · cytosol", "compartment_ids": [ "GO:0005782", "GO:0005829" ], "rhea_ids": [ "RHEA:25805" ], "source_url": "https://reactome.org/content/detail/R-HSA-141341", "summary": "SMOX-3 oxidises SPN to SPM is a Reactome release 97 curated reaction event. The source record connects SPN, H2O, O2 to SPM, 3APAL, H2O2 in peroxisomal matrix · cytosol and cross-references RHEA:25805.", "equation": "SPN + H2O + O2 → SPM + 3APAL + H2O2", "inputs": [ { "node_id": "R-ALL-141342", "reactome_id": "R-ALL-141342", "label": "SPN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:45725", "formula": "C10H30N4", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-141324", "reactome_id": "R-ALL-141324", "label": "SPM", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57834", "formula": "C7H22N3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141328", "reactome_id": "R-ALL-141328", "label": "3APAL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58374", "formula": "C3H8NO", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113575", "reactome_id": "R-ALL-113575", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "spermine oxidase activity of SMOX-3 [cytosol]", "activity_id": "5603125", "ec_numbers": [ "1.5.3.16" ], "accessions": null } ], "citations": [ { "id": "PMID:12141946", "title": "Identification and characterization of a novel flavin-containing spermine oxidase of mammalian cell origin", "journal": "Biochem J", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12141946/" }, { "id": "PMID:12727196", "title": "Properties of purified recombinant human polyamine oxidase, PAOh1/SMO", "journal": "Biochem Biophys Res Commun", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12727196/" } ], "x": 7461.333333333333, "y": 2900.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-141348", "label": "PAO:FAD oxidises NASPM to PTCN", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-one" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:25813" ], "source_url": "https://reactome.org/content/detail/R-HSA-141348", "summary": "PAO:FAD oxidises NASPM to PTCN is a Reactome release 97 curated reaction event. The source record connects NASPM, H2O, O2 to PTCN, H2O2, 3AAPNAL in peroxisomal matrix and cross-references RHEA:25813.", "equation": "NASPM + H2O + O2 → PTCN + H2O2 + 3AAPNAL", "inputs": [ { "node_id": "R-ALL-141349", "reactome_id": "R-ALL-141349", "label": "NASPM", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58324", "formula": "C9H23N3O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-141350", "reactome_id": "R-ALL-141350", "label": "PTCN", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:326268", "formula": "C4H14N2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113575", "reactome_id": "R-ALL-113575", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141327", "reactome_id": "R-ALL-141327", "label": "3AAPNAL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30322", "formula": "C5H9NO2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "N(1)-acetylpolyamine oxidase activity of PAOX:FAD [peroxisomal matrix]", "activity_id": "141347", "ec_numbers": [ "1.5.3.13" ], "accessions": null } ], "citations": [ { "id": "PMID:12477380", "title": "Genomic identification and biochemical characterization of the mammalian polyamine oxidase involved in polyamine back-conversion", "journal": "Biochem J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12477380/" } ], "x": 7740, "y": 2900.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-141351", "label": "PAOX:FAD oxidises NASPN to SPM", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-one" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:25801" ], "source_url": "https://reactome.org/content/detail/R-HSA-141351", "summary": "PAOX:FAD oxidises NASPN to SPM is a Reactome release 97 curated reaction event. The source record connects NASPN, H2O, O2 to SPM, H2O2, 3AAPNAL in peroxisomal matrix and cross-references RHEA:25801.", "equation": "NASPN + H2O + O2 → SPM + H2O2 + 3AAPNAL", "inputs": [ { "node_id": "R-ALL-141323", "reactome_id": "R-ALL-141323", "label": "NASPN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58101", "formula": "C12H31N4O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-141324", "reactome_id": "R-ALL-141324", "label": "SPM", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57834", "formula": "C7H22N3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113575", "reactome_id": "R-ALL-113575", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141327", "reactome_id": "R-ALL-141327", "label": "3AAPNAL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30322", "formula": "C5H9NO2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "N(1)-acetylpolyamine oxidase activity of PAOX:FAD [peroxisomal matrix]", "activity_id": "141347", "ec_numbers": [ "1.5.3.13" ], "accessions": null } ], "citations": [ { "id": "PMID:12477380", "title": "Genomic identification and biochemical characterization of the mammalian polyamine oxidase involved in polyamine back-conversion", "journal": "Biochem J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12477380/" } ], "x": 8018.666666666667, "y": 2900.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1475017", "label": "Carbonic anhydrase dehydrates bicarbonate (plasma membrane)", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "plasma membrane · extracellular region", "compartment_ids": [ "GO:0005886", "GO:0005576" ], "rhea_ids": [ "RHEA:10749" ], "source_url": "https://reactome.org/content/detail/R-HSA-1475017", "summary": "Carbonic anhydrase dehydrates bicarbonate (plasma membrane) is a Reactome release 97 curated reaction event. The source record connects H+, HCO3- to CO2, H2O in plasma membrane · extracellular region and cross-references RHEA:10749.", "equation": "H+ + HCO3- → CO2 + H2O", "inputs": [ { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-425425", "reactome_id": "R-ALL-425425", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1237009", "reactome_id": "R-ALL-1237009", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA4,9,14,12:Zn2+ [plasma membrane]", "activity_id": "1475013", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:2111324", "title": "Carbonic anhydrase IV from human lung. Purification, characterization, and comparison with membrane carbonic anhydrase from human kidney", "journal": "J Biol Chem", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2111324/" }, { "id": "PMID:7625839", "title": "Carbonic anhydrase IV: role of removal of C-terminal domain in glycosylphosphatidylinositol anchoring and realization of enzyme activity", "journal": "Arch Biochem Biophys", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7625839/" }, { "id": "PMID:15501038", "title": "Carbonic anhydrase inhibitors: inhibition of the membrane-bound human isozyme IV with anions", "journal": "Bioorg Med Chem Lett", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15501038/" }, { "id": "PMID:9054574", "title": "Catalysis and inhibition of human carbonic anhydrase IV", "journal": "Biochemistry", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9054574/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" }, { "id": "PMID:18703501", "title": "Biochemical characterization of CA IX, one of the most active carbonic anhydrase isozymes", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18703501/" } ], "x": 7078.666666666667, "y": 806.8333333333334, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1475022", "label": "Carbonic anhydrase dehydrates bicarbonate (cytosol)", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10749" ], "source_url": "https://reactome.org/content/detail/R-HSA-1475022", "summary": "Carbonic anhydrase dehydrates bicarbonate (cytosol) is a Reactome release 97 curated reaction event. The source record connects H+, HCO3- to CO2, H2O in cytosol and cross-references RHEA:10749.", "equation": "H+ + HCO3- → CO2 + H2O", "inputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111627", "reactome_id": "R-ALL-111627", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA1,2,3,7,13:Zinc [cytosol]", "activity_id": "1475014", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:4994926", "title": "The carbon dioxide hydration activity of carbonic anhydrase. I. Stop-flow kinetic studies on the native human isoenzymes B and C", "journal": "J Biol Chem", "year": 1971, "url": "https://pubmed.ncbi.nlm.nih.gov/4994926/" }, { "id": "PMID:6819139", "title": "A 13C nuclear magnetic resonance study of CO2/HCO-3 exchange catalyzed by human carbonic anhydrase I", "journal": "Eur J Biochem", "year": 1982, "url": "https://pubmed.ncbi.nlm.nih.gov/6819139/" }, { "id": "PMID:1554744", "title": "Buffer dependence of CO2 hydration catalyzed by human carbonic anhydrase I", "journal": "Biochim Biophys Acta", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1554744/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" }, { "id": "PMID:6433979", "title": "Anion inhibition of CO2 hydration catalyzed by human carbonic anhydrase II. Mechanistic implications", "journal": "Biochim Biophys Acta", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6433979/" }, { "id": "PMID:6407977", "title": "A chemical and enzymological comparison of the common major human erythrocyte carbonic anhydrase II, its minor component, and a new genetic variant, CA II Melbourne (237 Pro leads to His)", "journal": "Hum Genet", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/6407977/" } ], "x": 6521.333333333333, "y": 936.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1475025", "label": "Carbonic anhydrase hydrates carbon dioxide (plasma membrane)", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "plasma membrane · extracellular region", "compartment_ids": [ "GO:0005886", "GO:0005576" ], "rhea_ids": [ "RHEA:10750" ], "source_url": "https://reactome.org/content/detail/R-HSA-1475025", "summary": "Carbonic anhydrase hydrates carbon dioxide (plasma membrane) is a Reactome release 97 curated reaction event. The source record connects CO2, H2O to H+, HCO3- in plasma membrane · extracellular region and cross-references RHEA:10750.", "equation": "CO2 + H2O → H+ + HCO3-", "inputs": [ { "node_id": "R-ALL-1237009", "reactome_id": "R-ALL-1237009", "label": "CO2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-425425", "reactome_id": "R-ALL-425425", "label": "HCO3-", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA4,9,14,12:Zn2+ [plasma membrane]", "activity_id": "1475013", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:2111324", "title": "Carbonic anhydrase IV from human lung. Purification, characterization, and comparison with membrane carbonic anhydrase from human kidney", "journal": "J Biol Chem", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2111324/" }, { "id": "PMID:7625839", "title": "Carbonic anhydrase IV: role of removal of C-terminal domain in glycosylphosphatidylinositol anchoring and realization of enzyme activity", "journal": "Arch Biochem Biophys", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7625839/" }, { "id": "PMID:15501038", "title": "Carbonic anhydrase inhibitors: inhibition of the membrane-bound human isozyme IV with anions", "journal": "Bioorg Med Chem Lett", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15501038/" }, { "id": "PMID:9054574", "title": "Catalysis and inhibition of human carbonic anhydrase IV", "journal": "Biochemistry", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9054574/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" }, { "id": "PMID:18703501", "title": "Biochemical characterization of CA IX, one of the most active carbonic anhydrase isozymes", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18703501/" } ], "x": 6800, "y": 936.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1475026", "label": "Carbonic anhydrase hydrates carbon dioxide (cytosol)", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10750" ], "source_url": "https://reactome.org/content/detail/R-HSA-1475026", "summary": "Carbonic anhydrase hydrates carbon dioxide (cytosol) is a Reactome release 97 curated reaction event. The source record connects CO2, H2O to H+, HCO3- in cytosol and cross-references RHEA:10750.", "equation": "CO2 + H2O → H+ + HCO3-", "inputs": [ { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111627", "reactome_id": "R-ALL-111627", "label": "HCO3-", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA1,2,3,7,13:Zinc [cytosol]", "activity_id": "1475014", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:4994926", "title": "The carbon dioxide hydration activity of carbonic anhydrase. I. Stop-flow kinetic studies on the native human isoenzymes B and C", "journal": "J Biol Chem", "year": 1971, "url": "https://pubmed.ncbi.nlm.nih.gov/4994926/" }, { "id": "PMID:6819139", "title": "A 13C nuclear magnetic resonance study of CO2/HCO-3 exchange catalyzed by human carbonic anhydrase I", "journal": "Eur J Biochem", "year": 1982, "url": "https://pubmed.ncbi.nlm.nih.gov/6819139/" }, { "id": "PMID:1554744", "title": "Buffer dependence of CO2 hydration catalyzed by human carbonic anhydrase I", "journal": "Biochim Biophys Acta", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1554744/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" }, { "id": "PMID:6433979", "title": "Anion inhibition of CO2 hydration catalyzed by human carbonic anhydrase II. Mechanistic implications", "journal": "Biochim Biophys Acta", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6433979/" }, { "id": "PMID:6407977", "title": "A chemical and enzymological comparison of the common major human erythrocyte carbonic anhydrase II, its minor component, and a new genetic variant, CA II Melbourne (237 Pro leads to His)", "journal": "Hum Genet", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/6407977/" } ], "x": 7078.666666666667, "y": 936.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1475028", "label": "Carbonic anhydrase dehydrates bicarbonate (mitochondria)", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:10749" ], "source_url": "https://reactome.org/content/detail/R-HSA-1475028", "summary": "Carbonic anhydrase dehydrates bicarbonate (mitochondria) is a Reactome release 97 curated reaction event. The source record connects H+, HCO3- to CO2, H2O in mitochondrial matrix and cross-references RHEA:10749.", "equation": "H+ + HCO3- → CO2 + H2O", "inputs": [ { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29896", "reactome_id": "R-ALL-29896", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA5A,B:Zinc [mitochondrial matrix]", "activity_id": "1475031", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:16302824", "title": "Carbonic anhydrase inhibitors. The mitochondrial isozyme VB as a new target for sulfonamide and sulfamate inhibitors", "journal": "J Med Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16302824/" }, { "id": "PMID:14611844", "title": "Carbonic anhydrase inhibitors: inhibition of human and murine mitochondrial isozymes V with anions", "journal": "Bioorg Med Chem Lett", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14611844/" }, { "id": "PMID:17761422", "title": "Carbonic anhydrase inhibitors: the inhibition profiles of the human mitochondrial isoforms VA and VB with anions are very different", "journal": "Bioorg Med Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17761422/" }, { "id": "PMID:10409679", "title": "Human mitochondrial carbonic anhydrase VB. cDNA cloning, mRNA expression, subcellular localization, and mapping to chromosome x", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10409679/" }, { "id": "PMID:8356065", "title": "Human mitochondrial carbonic anhydrase: cDNA cloning, expression, subcellular localization, and mapping to chromosome 16", "journal": "Proc Natl Acad Sci U S A", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8356065/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" } ], "x": 6521.333333333333, "y": 1066.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1475032", "label": "Carbonic anhydrase hydrates carbon dioxide (mitochondria)", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "carbon-dioxide" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:10750" ], "source_url": "https://reactome.org/content/detail/R-HSA-1475032", "summary": "Carbonic anhydrase hydrates carbon dioxide (mitochondria) is a Reactome release 97 curated reaction event. The source record connects CO2, H2O to H+, HCO3- in mitochondrial matrix and cross-references RHEA:10750.", "equation": "CO2 + H2O → H+ + HCO3-", "inputs": [ { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29896", "reactome_id": "R-ALL-29896", "label": "HCO3-", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carbonate dehydratase activity of CA5A,B:Zinc [mitochondrial matrix]", "activity_id": "1475031", "ec_numbers": [ "4.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:16302824", "title": "Carbonic anhydrase inhibitors. The mitochondrial isozyme VB as a new target for sulfonamide and sulfamate inhibitors", "journal": "J Med Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16302824/" }, { "id": "PMID:14611844", "title": "Carbonic anhydrase inhibitors: inhibition of human and murine mitochondrial isozymes V with anions", "journal": "Bioorg Med Chem Lett", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14611844/" }, { "id": "PMID:17761422", "title": "Carbonic anhydrase inhibitors: the inhibition profiles of the human mitochondrial isoforms VA and VB with anions are very different", "journal": "Bioorg Med Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17761422/" }, { "id": "PMID:10409679", "title": "Human mitochondrial carbonic anhydrase VB. cDNA cloning, mRNA expression, subcellular localization, and mapping to chromosome x", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10409679/" }, { "id": "PMID:8356065", "title": "Human mitochondrial carbonic anhydrase: cDNA cloning, expression, subcellular localization, and mapping to chromosome 16", "journal": "Proc Natl Acad Sci U S A", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8356065/" }, { "id": "PMID:9336012", "title": "Structure and mechanism of carbonic anhydrase", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9336012/" } ], "x": 6800, "y": 1066.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1482533", "label": "2-acyl LPC is acylated to PC by LPCAT", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:10332" ], "source_url": "https://reactome.org/content/detail/R-HSA-1482533", "summary": "2-acyl LPC is acylated to PC by LPCAT is a Reactome release 97 curated reaction event. The source record connects 2-acyl LPC, acyl-CoA to PC, CoA-SH in endoplasmic reticulum membrane · cytosol and cross-references RHEA:10332.", "equation": "2-acyl LPC + acyl-CoA → PC + CoA-SH", "inputs": [ { "node_id": "R-ALL-975614", "reactome_id": "R-ALL-975614", "label": "2-acyl LPC", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16728", "formula": "C9H20NO7PR", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500578", "reactome_id": "R-ALL-1500578", "label": "acyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58342", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-426925", "reactome_id": "R-ALL-426925", "label": "PC", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57643", "formula": "C10H18NO8PR2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "2-acylglycerol-3-phosphate O-acyltransferase activity of LPCAT [endoplasmic reticulum membrane]", "activity_id": "1524043", "ec_numbers": [ "2.3.1.52" ], "accessions": null } ], "citations": [ { "id": "PMID:18458083", "title": "Molecular identification of a novel mammalian brain isoform of acyl-CoA:lysophospholipid acyltransferase with prominent ethanolamine lysophospholipid acylating activity, LPEAT2", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18458083/" }, { "id": "PMID:16243729", "title": "Cloning and characterization a novel human 1-acyl-sn-glycerol-3-phosphate acyltransferase gene AGPAT7", "journal": "DNA Seq", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16243729/" }, { "id": "PMID:18772128", "title": "Lysophospholipid acyltransferases and arachidonate recycling in human neutrophils", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18772128/" }, { "id": "PMID:16704971", "title": "Cloning and characterization of mouse lung-type acyl-CoA:lysophosphatidylcholine acyltransferase 1 (LPCAT1). Expression in alveolar type II cells and possible involvement in surfactant production", "journal": "J Biol Chem", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16704971/" }, { "id": "PMID:16864775", "title": "Identification and characterization of a lysophosphatidylcholine acyltransferase in alveolar type II cells", "journal": "Proc Natl Acad Sci U S A", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16864775/" }, { "id": "PMID:17182612", "title": "A single enzyme catalyzes both platelet-activating factor production and membrane biogenesis of inflammatory cells. Cloning and characterization of acetyl-CoA:LYSO-PAF acetyltransferase", "journal": "J Biol Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17182612/" } ], "x": 2938.666666666667, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1482543", "label": "2-MAG is hydrolyzed to fatty acid and glycerol by MGLL", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:44688" ], "source_url": "https://reactome.org/content/detail/R-HSA-1482543", "summary": "2-MAG is hydrolyzed to fatty acid and glycerol by MGLL is a Reactome release 97 curated reaction event. The source record connects 2-MAG, H2O to Glycerol, fatty acid in endoplasmic reticulum membrane · cytosol and cross-references RHEA:44688.", "equation": "2-MAG + H2O → Glycerol + fatty acid", "inputs": [ { "node_id": "R-ALL-1498728", "reactome_id": "R-ALL-1498728", "label": "2-MAG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17389", "formula": "C4H7O4R", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76116", "reactome_id": "R-ALL-76116", "label": "Glycerol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17754", "formula": "C3H8O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500593", "reactome_id": "R-ALL-1500593", "label": "fatty acid", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:35366", "formula": "CHO2R", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "monoacylglycerol lipase activity of MGLL dimer [endoplasmic reticulum membrane]", "activity_id": "1500582", "ec_numbers": [ "3.1.1.23" ], "accessions": null } ], "citations": [ { "id": "PMID:18452279", "title": "Full mass spectrometric characterization of human monoacylglycerol lipase generated by large-scale expression and single-step purification", "journal": "J Proteome Res", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18452279/" }, { "id": "PMID:15272052", "title": "RNA interference suggests a primary role for monoacylglycerol lipase in the degradation of the endocannabinoid 2-arachidonoylglycerol", "journal": "Mol Pharmacol", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15272052/" }, { "id": "PMID:19957260", "title": "Crystal structure of the human monoacylglycerol lipase, a key actor in endocannabinoid signaling", "journal": "Chembiochem", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/19957260/" }, { "id": "PMID:19962385", "title": "Structural basis for human monoglyceride lipase inhibition", "journal": "J Mol Biol", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/19962385/" } ], "x": 2381.3333333333335, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1482547", "label": "1-acyl LPC is acylated to PC by LPCAT", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:12937" ], "source_url": "https://reactome.org/content/detail/R-HSA-1482547", "summary": "1-acyl LPC is acylated to PC by LPCAT is a Reactome release 97 curated reaction event. The source record connects 1-acyl LPC, acyl-CoA to PC, CoA-SH in endoplasmic reticulum membrane · cytosol and cross-references RHEA:12937.", "equation": "1-acyl LPC + acyl-CoA → PC + CoA-SH", "inputs": [ { "node_id": "R-ALL-1524028", "reactome_id": "R-ALL-1524028", "label": "1-acyl LPC", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17504", "formula": "C9H20NO7PR", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500578", "reactome_id": "R-ALL-1500578", "label": "acyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58342", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-426925", "reactome_id": "R-ALL-426925", "label": "PC", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57643", "formula": "C10H18NO8PR2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "1-acylglycerol-3-phosphate O-acyltransferase activity of LPCAT [endoplasmic reticulum membrane]", "activity_id": "1524024", "ec_numbers": [ "2.3.1.51" ], "accessions": null } ], "citations": [ { "id": "PMID:18458083", "title": "Molecular identification of a novel mammalian brain isoform of acyl-CoA:lysophospholipid acyltransferase with prominent ethanolamine lysophospholipid acylating activity, LPEAT2", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18458083/" }, { "id": "PMID:16243729", "title": "Cloning and characterization a novel human 1-acyl-sn-glycerol-3-phosphate acyltransferase gene AGPAT7", "journal": "DNA Seq", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16243729/" }, { "id": "PMID:18772128", "title": "Lysophospholipid acyltransferases and arachidonate recycling in human neutrophils", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18772128/" }, { "id": "PMID:16704971", "title": "Cloning and characterization of mouse lung-type acyl-CoA:lysophosphatidylcholine acyltransferase 1 (LPCAT1). Expression in alveolar type II cells and possible involvement in surfactant production", "journal": "J Biol Chem", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16704971/" }, { "id": "PMID:16864775", "title": "Identification and characterization of a lysophosphatidylcholine acyltransferase in alveolar type II cells", "journal": "Proc Natl Acad Sci U S A", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16864775/" }, { "id": "PMID:17182612", "title": "A single enzyme catalyzes both platelet-activating factor production and membrane biogenesis of inflammatory cells. Cloning and characterization of acetyl-CoA:LYSO-PAF acetyltransferase", "journal": "J Biol Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17182612/" } ], "x": 2660, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1482598", "label": "1-acyl LPI is acylated to PI by MBOAT7", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:33195" ], "source_url": "https://reactome.org/content/detail/R-HSA-1482598", "summary": "1-acyl LPI is acylated to PI by MBOAT7 is a Reactome release 97 curated reaction event. The source record connects 1-acyl LPI, acyl-CoA to PI, CoA-SH in endoplasmic reticulum membrane · cytosol and cross-references RHEA:33195.", "equation": "1-acyl LPI + acyl-CoA → PI + CoA-SH", "inputs": [ { "node_id": "R-ALL-1498733", "reactome_id": "R-ALL-1498733", "label": "1-acyl LPI", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28914", "formula": "C10H18O12PR", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500578", "reactome_id": "R-ALL-1500578", "label": "acyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58342", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-426639", "reactome_id": "R-ALL-426639", "label": "PI", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57880", "formula": "C11H16O13PR2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "1-acylglycerol-3-phosphate O-acyltransferase activity of MBOAT7 [endoplasmic reticulum membrane]", "activity_id": "1524032", "ec_numbers": [ "2.3.1.51" ], "accessions": null } ], "citations": [ { "id": "PMID:18772128", "title": "Lysophospholipid acyltransferases and arachidonate recycling in human neutrophils", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18772128/" }, { "id": "PMID:18094042", "title": "Caenorhabditis elegans mboa-7, a member of the MBOAT family, is required for selective incorporation of polyunsaturated fatty acids into phosphatidylinositol", "journal": "Mol Biol Cell", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18094042/" } ], "x": 2938.666666666667, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1482626", "label": "2-acyl LPI is acylated to PI by MBOAT7", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:35271" ], "source_url": "https://reactome.org/content/detail/R-HSA-1482626", "summary": "2-acyl LPI is acylated to PI by MBOAT7 is a Reactome release 97 curated reaction event. The source record connects 2-acyl LPI, acyl-CoA to PI, CoA-SH in endoplasmic reticulum membrane · cytosol and cross-references RHEA:35271.", "equation": "2-acyl LPI + acyl-CoA → PI + CoA-SH", "inputs": [ { "node_id": "R-ALL-1498737", "reactome_id": "R-ALL-1498737", "label": "2-acyl LPI", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62746", "formula": "C10H18O12PR", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500578", "reactome_id": "R-ALL-1500578", "label": "acyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58342", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-426639", "reactome_id": "R-ALL-426639", "label": "PI", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57880", "formula": "C11H16O13PR2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "2-acylglycerol-3-phosphate O-acyltransferase activity of MBOAT7 [endoplasmic reticulum membrane]", "activity_id": "1524055", "ec_numbers": [ "2.3.1.52" ], "accessions": null } ], "citations": [ { "id": "PMID:18772128", "title": "Lysophospholipid acyltransferases and arachidonate recycling in human neutrophils", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18772128/" }, { "id": "PMID:18094042", "title": "Caenorhabditis elegans mboa-7, a member of the MBOAT family, is required for selective incorporation of polyunsaturated fatty acids into phosphatidylinositol", "journal": "Mol Biol Cell", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18094042/" } ], "x": 2381.3333333333335, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1482636", "label": "1-acyl LPS is acylated to PS by LPSAT", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:33191" ], "source_url": "https://reactome.org/content/detail/R-HSA-1482636", "summary": "1-acyl LPS is acylated to PS by LPSAT is a Reactome release 97 curated reaction event. The source record connects 1-acyl LPS, acyl-CoA to PS, CoA-SH in endoplasmic reticulum membrane · cytosol and cross-references RHEA:33191.", "equation": "1-acyl LPS + acyl-CoA → PS + CoA-SH", "inputs": [ { "node_id": "R-ALL-1498724", "reactome_id": "R-ALL-1498724", "label": "1-acyl LPS", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:52603", "formula": "C7H13NO9PR", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500578", "reactome_id": "R-ALL-1500578", "label": "acyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58342", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1500646", "reactome_id": "R-ALL-1500646", "label": "PS", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:11750", "formula": "C8H12NO10PR2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "1-acylglycerol-3-phosphate O-acyltransferase activity of LPSAT [endoplasmic reticulum membrane]", "activity_id": "1524041", "ec_numbers": [ "2.3.1.51" ], "accessions": null } ], "citations": [ { "id": "PMID:18287005", "title": "Discovery of a lysophospholipid acyltransferase family essential for membrane asymmetry and diversity", "journal": "Proc Natl Acad Sci U S A", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18287005/" }, { "id": "PMID:18772128", "title": "Lysophospholipid acyltransferases and arachidonate recycling in human neutrophils", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18772128/" }, { "id": "PMID:18458083", "title": "Molecular identification of a novel mammalian brain isoform of acyl-CoA:lysophospholipid acyltransferase with prominent ethanolamine lysophospholipid acylating activity, LPEAT2", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18458083/" } ], "x": 2660, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1482695", "label": "G3P is acylated to 1-acyl LPA by GPAM/GPAT2 (OM)", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "mitochondrial outer membrane · cytosol", "compartment_ids": [ "GO:0005741", "GO:0005829" ], "rhea_ids": [ "RHEA:15325" ], "source_url": "https://reactome.org/content/detail/R-HSA-1482695", "summary": "G3P is acylated to 1-acyl LPA by GPAM/GPAT2 (OM) is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects G3P, acyl-CoA to 1-acyl LPA, CoA-SH in mitochondrial outer membrane · cytosol and cross-references RHEA:15325.", "equation": "G3P + acyl-CoA → 1-acyl LPA + CoA-SH", "inputs": [ { "node_id": "R-ALL-76114", "reactome_id": "R-ALL-76114", "label": "G3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57597", "formula": "C3H7O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500578", "reactome_id": "R-ALL-1500578", "label": "acyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58342", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1500580", "reactome_id": "R-ALL-1500580", "label": "1-acyl LPA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16975", "formula": "C4H8O7PR", "compartment": "mitochondrial outer membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycerol-3-phosphate O-acyltransferase activity of GPAM/GPAT2 [mitochondrial outer membrane]", "activity_id": "1500616", "ec_numbers": [ "2.3.1.15" ], "accessions": null } ], "citations": [ { "id": "PMID:18238778", "title": "AGPAT6 is a novel microsomal glycerol-3-phosphate acyltransferase", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18238778/" }, { "id": "PMID:18718904", "title": "Acyl-CoA:lysophospholipid acyltransferases", "journal": "J Biol Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/18718904/" }, { "id": "PMID:19336658", "title": "Biochemistry, physiology, and genetics of GPAT, AGPAT, and lipin enzymes in triglyceride synthesis", "journal": "Am J Physiol Endocrinol Metab", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19336658/" } ], "x": 2938.666666666667, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-1497869", "label": "Salvage - Sepiapterin is reduced to q-BH2", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18955" ], "source_url": "https://reactome.org/content/detail/R-HSA-1497869", "summary": "Salvage - Sepiapterin is reduced to q-BH2 is a Reactome release 97 curated reaction event. The source record connects NADPH, sepiapterin, H+ to q-BH2, NADP+ in cytosol and cross-references RHEA:18955.", "equation": "NADPH + sepiapterin + H+ → q-BH2 + NADP+", "inputs": [ { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-1497811", "reactome_id": "R-ALL-1497811", "label": "sepiapterin", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:194527", "formula": "C9H11N5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-1497857", "reactome_id": "R-ALL-1497857", "label": "q-BH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43029", "formula": "C9H13N5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "sepiapterin reductase (NADP+) activity of p-SPR dimer [cytosol]", "activity_id": "1497839", "ec_numbers": [ "1.1.1.153" ], "accessions": null } ], "citations": [ { "id": "PMID:18511317", "title": "Cellular uptake of sepiapterin and push-pull accumulation of tetrahydrobiopterin", "journal": "Mol Genet Metab", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18511317/" } ], "x": 4451.333333333333, "y": 3622.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-159443", "label": "benzoate + Coenzyme A + ATP =\u003e benzoyl-CoA + AMP + pyrophosphate", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-two" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:10133" ], "source_url": "https://reactome.org/content/detail/R-HSA-159443", "summary": "benzoate + Coenzyme A + ATP =\u003e benzoyl-CoA + AMP + pyrophosphate is a Reactome release 97 curated reaction event. The source record connects BENZA, ATP, CoA-SH to BEZ-CoA, AMP, PPi in mitochondrial matrix and cross-references RHEA:10133.", "equation": "BENZA + ATP + CoA-SH → BEZ-CoA + AMP + PPi", "inputs": [ { "node_id": "R-ALL-159446", "reactome_id": "R-ALL-159446", "label": "BENZA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30746", "formula": "C7H6O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-159447", "reactome_id": "R-ALL-159447", "label": "BEZ-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57369", "formula": "C28H36N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159448", "reactome_id": "R-ALL-159448", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "CoA-ligase activity of xenobiotic/medium-chain fatty acid:CoA ligases [mitochondrial matrix]", "activity_id": "177146", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:12616642", "title": "Isolation, sequencing, and expression of a cDNA for the HXM-A form of xenobiotic/medium-chain fatty acid:CoA ligase from human liver mitochondria", "journal": "J Biochem Mol Toxicol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12616642/" }, { "id": "PMID:10434065", "title": "Characterization of the CoA ligases of human liver mitochondria catalyzing the activation of short- and medium-chain fatty acids and xenobiotic carboxylic acids", "journal": "Biochim Biophys Acta", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10434065/" } ], "x": 7740, "y": 3476.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-159567", "label": "ACSM2B-like proteins transform ST to ST-CoA", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-two" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:61669" ], "source_url": "https://reactome.org/content/detail/R-HSA-159567", "summary": "ACSM2B-like proteins transform ST to ST-CoA is a Reactome release 97 curated reaction event. The source record connects ST, ATP, CoA-SH to ST-CoA, AMP, PPi in mitochondrial matrix and cross-references RHEA:61669.", "equation": "ST + ATP + CoA-SH → ST-CoA + AMP + PPi", "inputs": [ { "node_id": "R-ALL-159559", "reactome_id": "R-ALL-159559", "label": "ST", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30762", "formula": "C7H5O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-159557", "reactome_id": "R-ALL-159557", "label": "ST-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:32587", "formula": "C28H40N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159448", "reactome_id": "R-ALL-159448", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "CoA-ligase activity of ACSM2B-like proteins [mitochondrial matrix]", "activity_id": "159441", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:5862532", "title": "Pharmacokinetics of salicylate elimination in man", "journal": "J Pharm Sci", "year": 1965, "url": "https://pubmed.ncbi.nlm.nih.gov/5862532/" }, { "id": "PMID:12616642", "title": "Isolation, sequencing, and expression of a cDNA for the HXM-A form of xenobiotic/medium-chain fatty acid:CoA ligase from human liver mitochondria", "journal": "J Biochem Mol Toxicol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12616642/" } ], "x": 8018.666666666667, "y": 3476.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1614544", "label": "Sulfite is oxidized to sulfate", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:24601" ], "source_url": "https://reactome.org/content/detail/R-HSA-1614544", "summary": "Sulfite is oxidized to sulfate is a Reactome release 97 curated reaction event. The source record connects sulfite(2-), O2, H2O to SO4(2-), H2O2 in mitochondrial matrix and cross-references RHEA:24601.", "equation": "sulfite(2-) + O2 + H2O → SO4(2-) + H2O2", "inputs": [ { "node_id": "R-ALL-1614538", "reactome_id": "R-ALL-1614538", "label": "sulfite(2-)", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17359", "formula": "O3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113533", "reactome_id": "R-ALL-113533", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-1614584", "reactome_id": "R-ALL-1614584", "label": "SO4(2-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16189", "formula": "O4S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1614617", "reactome_id": "R-ALL-1614617", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "sulfite oxidase activity of holo-SUOX [mitochondrial matrix]", "activity_id": "1614541", "ec_numbers": [ "1.8.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:16475804", "title": "The G473D mutation impairs dimerization and catalysis in human sulfite oxidase", "journal": "Biochemistry", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16475804/" }, { "id": "PMID:17459792", "title": "Sulfite oxidizing enzymes", "journal": "Biochim Biophys Acta", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17459792/" } ], "x": 1808.6666666666667, "y": 2917.6666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1614583", "label": "PXLP-K212-CTH cleaves L-Cystathionine", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14006" ], "source_url": "https://reactome.org/content/detail/R-HSA-1614583", "summary": "PXLP-K212-CTH cleaves L-Cystathionine is a Reactome release 97 curated reaction event. The source record connects L-Cystathionine, H2O to L-Cys, 2OBUTA, NH4+ in cytosol and cross-references RHEA:14006.", "equation": "L-Cystathionine + H2O → L-Cys + 2OBUTA + NH4+", "inputs": [ { "node_id": "R-ALL-1614518", "reactome_id": "R-ALL-1614518", "label": "L-Cystathionine", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58161", "formula": "C7H14N2O4S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111707", "reactome_id": "R-ALL-111707", "label": "L-Cys", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:35235", "formula": "C3H7NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-936714", "reactome_id": "R-ALL-936714", "label": "2OBUTA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16763", "formula": "C4H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "cystathionine gamma-lyase activity of PXLP-K212-CTH tetramer [cytosol]", "activity_id": "1614601", "ec_numbers": [ "4.4.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:19261609", "title": "H2S biogenesis by human cystathionine gamma-lyase leads to the novel sulfur metabolites lanthionine and homolanthionine and is responsive to the grade of hyperhomocysteinemia", "journal": "J Biol Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19261609/" }, { "id": "PMID:10212249", "title": "Kinetics and inhibition of recombinant human cystathionine gamma-lyase. Toward the rational control of transsulfuration", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10212249/" } ], "x": 1251.3333333333333, "y": 2994.1111111111113, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1614614", "label": "Cysteine is degraded to pyruvate and H2S", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24932" ], "source_url": "https://reactome.org/content/detail/R-HSA-1614614", "summary": "Cysteine is degraded to pyruvate and H2S is a Reactome release 97 curated reaction event. The source record connects H2O, L-Cys to H+, NH4+, PYR in cytosol and cross-references RHEA:24932.", "equation": "H2O + L-Cys → H+ + NH4+ + PYR + H2S", "inputs": [ { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111707", "reactome_id": "R-ALL-111707", "label": "L-Cys", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:35235", "formula": "C3H7NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1614532", "reactome_id": "R-ALL-1614532", "label": "H2S", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29919", "formula": "HS", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carbon-sulfur lyase activity of PXLP-K212-CTH tetramer [cytosol]", "activity_id": "1614529", "ec_numbers": [ "4.4" ], "accessions": null } ], "citations": [ { "id": "PMID:19261609", "title": "H2S biogenesis by human cystathionine gamma-lyase leads to the novel sulfur metabolites lanthionine and homolanthionine and is responsive to the grade of hyperhomocysteinemia", "journal": "J Biol Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19261609/" }, { "id": "PMID:10212249", "title": "Kinetics and inhibition of recombinant human cystathionine gamma-lyase. Toward the rational control of transsulfuration", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10212249/" } ], "x": 1530, "y": 2841.222222222222, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1614631", "label": "Homocysteine is degraded to oxobutanoate and H2S", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14502" ], "source_url": "https://reactome.org/content/detail/R-HSA-1614631", "summary": "Homocysteine is degraded to oxobutanoate and H2S is a Reactome release 97 curated reaction event. The source record connects HCYS, H2O to 2OBUTA, H2S, NH4+ in cytosol and cross-references RHEA:14502.", "equation": "HCYS + H2O → 2OBUTA + H2S + NH4+ + H+", "inputs": [ { "node_id": "R-ALL-174369", "reactome_id": "R-ALL-174369", "label": "HCYS", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58199", "formula": "C4H9NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-936714", "reactome_id": "R-ALL-936714", "label": "2OBUTA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16763", "formula": "C4H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1614532", "reactome_id": "R-ALL-1614532", "label": "H2S", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29919", "formula": "HS", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "homocysteine desulfhydrase activity of PXLP-K212-CTH tetramer [cytosol]", "activity_id": "1614534", "ec_numbers": [ "4.4.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:19261609", "title": "H2S biogenesis by human cystathionine gamma-lyase leads to the novel sulfur metabolites lanthionine and homolanthionine and is responsive to the grade of hyperhomocysteinemia", "journal": "J Biol Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19261609/" }, { "id": "PMID:10212249", "title": "Kinetics and inhibition of recombinant human cystathionine gamma-lyase. Toward the rational control of transsulfuration", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10212249/" } ], "x": 1808.6666666666667, "y": 2841.222222222222, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-163213", "label": "Transfer of electrons through the succinate dehydrogenase complex", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport" ], "compartment": "mitochondrial inner membrane", "compartment_ids": [ "GO:0005743" ], "rhea_ids": [ "RHEA:13714" ], "source_url": "https://reactome.org/content/detail/R-HSA-163213", "summary": "Transfer of electrons through the succinate dehydrogenase complex is a Reactome release 97 curated reaction event. The source record connects CoQ, SUCCA to FUMA, CoQH2 in mitochondrial inner membrane and cross-references RHEA:13714.", "equation": "CoQ + SUCCA → FUMA + CoQH2", "inputs": [ { "node_id": "R-ALL-73663", "reactome_id": "R-ALL-73663", "label": "CoQ", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46245", "formula": "C59H90O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113536", "reactome_id": "R-ALL-113536", "label": "SUCCA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30031", "formula": "C4H4O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113588", "reactome_id": "R-ALL-113588", "label": "FUMA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29806", "formula": "C4H2O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162198", "reactome_id": "R-ALL-2162198", "label": "CoQH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:64183", "formula": "C59H92O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "succinate dehydrogenase (quinone) activity of SDH complex [mitochondrial inner membrane]", "activity_id": "70993", "ec_numbers": [ "1.3.5.1" ], "accessions": null } ], "citations": [ { "id": "PMID:23747254", "title": "The mitochondrial chaperone TRAP1 promotes neoplastic growth by inhibiting succinate dehydrogenase", "journal": "Cell Metab.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23747254/" }, { "id": "PMID:23564345", "title": "Molecular chaperone TRAP1 regulates a metabolic switch between mitochondrial respiration and aerobic glycolysis", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23564345/" }, { "id": "PMID:25564869", "title": "Inhibition of succinate dehydrogenase by the mitochondrial chaperone TRAP1 has anti-oxidant and anti-apoptotic effects on tumor cells", "journal": "Oncotarget", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/25564869/" } ], "x": 4451.333333333333, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-163214", "label": "Electron transfer from reduced cytochrome c to molecular oxygen", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport" ], "compartment": "mitochondrial inner membrane · mitochondrial intermembrane space · mitochondrial matrix", "compartment_ids": [ "GO:0005743", "GO:0005758", "GO:0005759" ], "rhea_ids": [ "RHEA:11437" ], "source_url": "https://reactome.org/content/detail/R-HSA-163214", "summary": "Electron transfer from reduced cytochrome c to molecular oxygen is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects O2, H+, Cytochrome c (reduced) to H2O, H+, Cytochrome c (oxidised) in mitochondrial inner membrane · mitochondrial intermembrane space · mitochondrial matrix and cross-references RHEA:11437.", "equation": "O2 + 12 H+ + 4 Cytochrome c (reduced) → 2 H2O + 8 H+ + 4 Cytochrome c (oxidised)", "inputs": [ { "node_id": "R-ALL-113533", "reactome_id": "R-ALL-113533", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 12, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-352609", "reactome_id": "R-HSA-352609", "label": "Cytochrome c (reduced)", "role": "input", "stoichiometry": 4, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-163953", "reactome_id": "R-ALL-163953", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-352607", "reactome_id": "R-HSA-352607", "label": "Cytochrome c (oxidised)", "role": "output", "stoichiometry": 4, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "catalysts": [ { "label": "cytochrome-c oxidase activity of Cytochrome c oxidase [mitochondrial inner membrane]", "activity_id": "164322", "ec_numbers": [ "7.1.1.9" ], "accessions": null } ], "citations": [ { "id": "PMID:7979252", "title": "Energy transduction by cytochrome complexes in mitochondrial and bacterial", "journal": "Annu Rev Biochem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7979252/" }, { "id": "PMID:11340051", "title": "Structures and proton-pumping strategies of mitochondrial respiratory", "journal": "Annu Rev Biophys Biomol Struct", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11340051/" }, { "id": "PMID:15223", "title": "Proton pump coupled to cytochrome c oxidase in mitochondria", "journal": "Nature", "year": 1977, "url": "https://pubmed.ncbi.nlm.nih.gov/15223/" }, { "id": "PMID:16760263", "title": "Assembly of mitochondrial cytochrome c-oxidase, a complicated and highly regulated cellular process", "journal": "Am. J. Physiol., Cell Physiol.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16760263/" }, { "id": "PMID:21958598", "title": "Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core", "journal": "Biochim. Biophys. Acta", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/21958598/" }, { "id": "PMID:17298220", "title": "Biogenesis of eukaryotic cytochrome c oxidase", "journal": "Physiol Res", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17298220/" } ], "x": 4730, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-163217", "label": "Complex I oxidises NADH to NAD+, reduces CoQ to CoQH2", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport" ], "compartment": "mitochondrial inner membrane", "compartment_ids": [ "GO:0005743" ], "rhea_ids": [ "RHEA:29092" ], "source_url": "https://reactome.org/content/detail/R-HSA-163217", "summary": "Complex I oxidises NADH to NAD+, reduces CoQ to CoQH2 is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects NADH, H+, CoQ to NAD+, H+, CoQH2 in mitochondrial inner membrane and cross-references RHEA:29092.", "equation": "NADH + 5 H+ + CoQ → NAD+ + 4 H+ + CoQH2", "inputs": [ { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 5, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-73663", "reactome_id": "R-ALL-73663", "label": "CoQ", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46245", "formula": "C59H90O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-163953", "reactome_id": "R-ALL-163953", "label": "H+", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-2162198", "reactome_id": "R-ALL-2162198", "label": "CoQH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:64183", "formula": "C59H92O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "NADH dehydrogenase (ubiquinone) activity of Complex I [mitochondrial inner membrane]", "activity_id": "164321", "ec_numbers": [ "7.1.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:9878551", "title": "cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase:", "journal": "Biochem Biophys Res Commun", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9878551/" }, { "id": "PMID:12837546", "title": "The nuclear encoded subunits of complex I from bovine heart mitochondria", "journal": "Biochim Biophys Acta", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12837546/" }, { "id": "PMID:15250827", "title": "Structural organization of mitochondrial human complex I: role of the ND4", "journal": "Biochem J", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15250827/" }, { "id": "PMID:14741580", "title": "The gross structure of the respiratory complex I: a Lego System", "journal": "Biochim Biophys Acta", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/14741580/" }, { "id": "PMID:11695836", "title": "Human NADH:ubiquinone oxidoreductase", "journal": "J Bioenerg Biomembr", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11695836/" }, { "id": "PMID:12231006", "title": "The energy-transducing NADH: quinone oxidoreductase, complex I", "journal": "Mol Aspects Med", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12231006/" } ], "x": 5008.666666666667, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-163402", "label": "diacylglycerol + H2O -\u003e 2-acylglycerol + fatty acid", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol · lipid droplet", "compartment_ids": [ "GO:0005829", "GO:0005811" ], "rhea_ids": [ "RHEA:33276" ], "source_url": "https://reactome.org/content/detail/R-HSA-163402", "summary": "diacylglycerol + H2O -\u003e 2-acylglycerol + fatty acid is a Reactome release 97 curated reaction event. The source record connects DAG, H2O to 2-acylglycerol, LCFA(-), H+ in cytosol · lipid droplet and cross-references RHEA:33276.", "equation": "DAG + H2O → 2-acylglycerol + LCFA(-) + H+", "inputs": [ { "node_id": "R-ALL-163414", "reactome_id": "R-ALL-163414", "label": "DAG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-163580", "reactome_id": "R-ALL-163580", "label": "2-acylglycerol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17389", "formula": "C4H7O4R", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8876730", "reactome_id": "R-ALL-8876730", "label": "LCFA(-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57560", "formula": "CO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "lipase activity of phosphorylated HSL dimer:FABP4 complex [lipid droplet]", "activity_id": "163604", "ec_numbers": [ "3.1.1" ], "accessions": null } ], "citations": [], "x": 3321.3333333333335, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-163432", "label": "cholesterol ester + H2O -\u003e cholesterol + fatty acid", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol · lipid droplet", "compartment_ids": [ "GO:0005829", "GO:0005811" ], "rhea_ids": [ "RHEA:36404" ], "source_url": "https://reactome.org/content/detail/R-HSA-163432", "summary": "cholesterol ester + H2O -\u003e cholesterol + fatty acid is a Reactome release 97 curated reaction event. The source record connects CHEST, H2O to CHOL, LCFA(-), H+ in cytosol · lipid droplet and cross-references RHEA:36404.", "equation": "CHEST + H2O → CHOL + LCFA(-) + H+", "inputs": [ { "node_id": "R-ALL-163579", "reactome_id": "R-ALL-163579", "label": "CHEST", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17002", "formula": "C28H45O2R", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-163586", "reactome_id": "R-ALL-163586", "label": "CHOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16113", "formula": "C27H46O", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8876730", "reactome_id": "R-ALL-8876730", "label": "LCFA(-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57560", "formula": "CO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "lipase activity of phosphorylated HSL dimer:FABP4 complex [lipid droplet]", "activity_id": "163604", "ec_numbers": [ "3.1.1" ], "accessions": null } ], "citations": [], "x": 3600, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-163551", "label": "triacylglycerol + H2O -\u003e diacylglycerol + fatty acid", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol · lipid droplet", "compartment_ids": [ "GO:0005829", "GO:0005811" ], "rhea_ids": [ "RHEA:12045" ], "source_url": "https://reactome.org/content/detail/R-HSA-163551", "summary": "triacylglycerol + H2O -\u003e diacylglycerol + fatty acid is a Reactome release 97 curated reaction event. The source record connects TAG, H2O to DAG, LCFA(-), H+ in cytosol · lipid droplet and cross-references RHEA:12045.", "equation": "TAG + H2O → DAG + LCFA(-) + H+", "inputs": [ { "node_id": "R-ALL-163438", "reactome_id": "R-ALL-163438", "label": "TAG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17855", "formula": "C6H5O6R3", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-163414", "reactome_id": "R-ALL-163414", "label": "DAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8876730", "reactome_id": "R-ALL-8876730", "label": "LCFA(-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57560", "formula": "CO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "triacylglycerol lipase activity of phosphorylated HSL dimer:FABP4 complex [lipid droplet]", "activity_id": "163447", "ec_numbers": [ "3.1.1.3" ], "accessions": null } ], "citations": [], "x": 3878.666666666667, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-163595", "label": "2-acylglycerol + H2O -\u003e glycerol + fatty acid", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol · lipid droplet", "compartment_ids": [ "GO:0005829", "GO:0005811" ], "rhea_ids": [ "RHEA:44689" ], "source_url": "https://reactome.org/content/detail/R-HSA-163595", "summary": "2-acylglycerol + H2O -\u003e glycerol + fatty acid is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects 2-acylglycerol, H2O to Glycerol, LCFA(-), H+ in cytosol · lipid droplet and cross-references RHEA:44689.", "equation": "2-acylglycerol + H2O → Glycerol + LCFA(-) + H+", "inputs": [ { "node_id": "R-ALL-163580", "reactome_id": "R-ALL-163580", "label": "2-acylglycerol", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17389", "formula": "C4H7O4R", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76116", "reactome_id": "R-ALL-76116", "label": "Glycerol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17754", "formula": "C3H8O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8876730", "reactome_id": "R-ALL-8876730", "label": "LCFA(-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57560", "formula": "CO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "monoacylglycerol lipase activity of MGLL [lipid droplet]", "activity_id": "163500", "ec_numbers": [ "3.1.1.23" ], "accessions": null } ], "citations": [], "x": 3321.3333333333335, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-163751", "label": "D-glyceraldehyde 3-phosphate + D-fructose 6-phosphate xylulose 5-phosphate + D-erythrose 4-phosphate", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:27628" ], "source_url": "https://reactome.org/content/detail/R-HSA-163751", "summary": "D-glyceraldehyde 3-phosphate + D-fructose 6-phosphate xylulose 5-phosphate + D-erythrose 4-phosphate is a Reactome release 97 curated reaction event. The source record connects GA3P, Fru(6)P to XY5P, E4P in cytosol and cross-references RHEA:27628.", "equation": "GA3P + Fru(6)P → XY5P + E4P", "inputs": [ { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29790", "reactome_id": "R-ALL-29790", "label": "XY5P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57737", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29878", "reactome_id": "R-ALL-29878", "label": "E4P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16897", "formula": "C4H7O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "transketolase activity of TKT dimer [cytosol]", "activity_id": "71323", "ec_numbers": [ "2.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:9357955", "title": "Aspartate 155 of human transketolase is essential for thiamine diphosphate-magnesium binding, and cofactor binding is required for dimer formation", "journal": "Biochim Biophys Acta", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9357955/" } ], "x": 590, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-163764", "label": "D-fructose 6-phosphate + D-erythrose 4-phosphate sedoheptulose 7-phosphate + D-glyceraldehyde 3-phosphate", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17055" ], "source_url": "https://reactome.org/content/detail/R-HSA-163764", "summary": "D-fructose 6-phosphate + D-erythrose 4-phosphate sedoheptulose 7-phosphate + D-glyceraldehyde 3-phosphate is a Reactome release 97 curated reaction event. The source record connects Fru(6)P, E4P to SH7P, GA3P in cytosol and cross-references RHEA:17055.", "equation": "Fru(6)P + E4P → SH7P + GA3P", "inputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29878", "reactome_id": "R-ALL-29878", "label": "E4P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16897", "formula": "C4H7O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29882", "reactome_id": "R-ALL-29882", "label": "SH7P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57483", "formula": "C7H13O10P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "transaldolase activity of TALDO1 dimer [cytosol]", "activity_id": "71333", "ec_numbers": [ "2.2.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:8300619", "title": "Cloning and expression of the human gene for transaldolase. A novel highly repetitive element constitutes an integral part of the coding sequence", "journal": "J Biol Chem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8300619/" }, { "id": "PMID:10869557", "title": "The three-dimensional structure of human transaldolase", "journal": "FEBS Lett.", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10869557/" }, { "id": "PMID:11283793", "title": "Transaldolase deficiency: liver cirrhosis associated with a new inborn error in the pentose phosphate pathway.", "journal": "Am J Hum Genet", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11283793/" } ], "x": 868.6666666666667, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1638104", "label": "UGCG transfers glucose to ceramide", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "Golgi membrane · Golgi lumen", "compartment_ids": [ "GO:0000139", "GO:0005796" ], "rhea_ids": [ "RHEA:12088" ], "source_url": "https://reactome.org/content/detail/R-HSA-1638104", "summary": "UGCG transfers glucose to ceramide is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, CERA-d18 to GlcCer, UDP, H+ in Golgi membrane · Golgi lumen and cross-references RHEA:12088.", "equation": "UDP-Glc + CERA-d18 → GlcCer + UDP + H+", "inputs": [ { "node_id": "R-ALL-744227", "reactome_id": "R-ALL-744227", "label": "UDP-Glc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9844975", "reactome_id": "R-ALL-9844975", "label": "CERA-d18", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:52639", "formula": "C19H36NO3R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1660674", "reactome_id": "R-ALL-1660674", "label": "GlcCer", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:22801", "formula": "C25H46NO8R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-205687", "reactome_id": "R-ALL-205687", "label": "UDP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-425969", "reactome_id": "R-ALL-425969", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ceramide glucosyltransferase activity of UGCG [Golgi membrane]", "activity_id": "1638095", "ec_numbers": [ "2.4.1.80" ], "accessions": null } ], "citations": [ { "id": "PMID:8643456", "title": "Expression cloning of a cDNA for human ceramide glucosyltransferase that catalyzes the first glycosylation step of glycosphingolipid synthesis", "journal": "Proc Natl Acad Sci U S A", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8643456/" }, { "id": "PMID:15799713", "title": "The reconstituted P-glycoprotein multidrug transporter is a flippase for glucosylceramide and other simple glycosphingolipids", "journal": "Biochem J", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15799713/" }, { "id": "PMID:14662772", "title": "Role of multiple drug resistance protein 1 in neutral but not acidic glycosphingolipid biosynthesis", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/14662772/" }, { "id": "PMID:23290777", "title": "Ceramide glycosylation catalyzed by glucosylceramide synthase and cancer drug resistance", "journal": "Adv. Cancer Res.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23290777/" } ], "x": 3878.666666666667, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-164651", "label": "Electron transfer from ubiquinol to cytochrome c of complex III", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport" ], "compartment": "mitochondrial inner membrane", "compartment_ids": [ "GO:0005743" ], "rhea_ids": [ "RHEA:11485" ], "source_url": "https://reactome.org/content/detail/R-HSA-164651", "summary": "Electron transfer from ubiquinol to cytochrome c of complex III is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects CoQ, H+, Cytochrome c (oxidised) to CoQ, CoQH2, H+ in mitochondrial inner membrane and cross-references RHEA:11485.", "equation": "CoQ + 2 H+ + 2 Cytochrome c (oxidised) + 2 CoQH2 → 2 CoQ + CoQH2 + 4 H+ + 2 Cytochrome c (reduced)", "inputs": [ { "node_id": "R-ALL-73663", "reactome_id": "R-ALL-73663", "label": "CoQ", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46245", "formula": "C59H90O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-352607", "reactome_id": "R-HSA-352607", "label": "Cytochrome c (oxidised)", "role": "input", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "EntityWithAccessionedSequence", "currency": false }, { "node_id": "R-ALL-2162198", "reactome_id": "R-ALL-2162198", "label": "CoQH2", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:64183", "formula": "C59H92O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-73663", "reactome_id": "R-ALL-73663", "label": "CoQ", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:46245", "formula": "C59H90O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162198", "reactome_id": "R-ALL-2162198", "label": "CoQH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:64183", "formula": "C59H92O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-163953", "reactome_id": "R-ALL-163953", "label": "H+", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-352609", "reactome_id": "R-HSA-352609", "label": "Cytochrome c (reduced)", "role": "output", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "catalysts": [ { "label": "quinol-cytochrome-c reductase activity of Cytochrome c reductase [mitochondrial inner membrane]", "activity_id": "164660", "ec_numbers": [ "7.1.1.8" ], "accessions": null } ], "citations": [ { "id": "PMID:186667", "title": "Possible molecular mechanisms of the protonmotive function of cytochrome", "journal": "J Theor Biol", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/186667/" }, { "id": "PMID:239860", "title": "Protonmotive redox mechanism of the cytochrome b-c1 complex in the", "journal": "FEBS Lett", "year": 1975, "url": "https://pubmed.ncbi.nlm.nih.gov/239860/" } ], "x": 5008.666666666667, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-164832", "label": "ATPase synthesizes ATP", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "chemiosmosis" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:57722" ], "source_url": "https://reactome.org/content/detail/R-HSA-164832", "summary": "ATPase synthesizes ATP is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects ATPase:ADP:Pi to ATPase:ATP, H2O in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:57722.", "equation": "ATPase:ADP:Pi → ATPase:ATP + H2O", "inputs": [ { "node_id": "R-HSA-164838", "reactome_id": "R-HSA-164838", "label": "ATPase:ADP:Pi", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-HSA-164835", "reactome_id": "R-HSA-164835", "label": "ATPase:ATP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "proton-transporting ATP synthase activity, rotational mechanism of ATPase:ADP:Pi [mitochondrial inner membrane]", "activity_id": "164536", "ec_numbers": [ "7.1.2.2" ], "accessions": null } ], "citations": [ { "id": "PMID:4517936", "title": "A new concept for energy coupling in oxidative phosphorylation based on a", "journal": "Proc Natl Acad Sci U S A", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4517936/" } ], "x": 5670, "y": 936.5, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-164834", "label": "Enzyme-bound ATP is released", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "chemiosmosis" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:57722" ], "source_url": "https://reactome.org/content/detail/R-HSA-164834", "summary": "Enzyme-bound ATP is released is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects ATPase:ATP, H+ to F1Fo ATP synthase, ATP, H+ in mitochondrial matrix and cross-references RHEA:57722.", "equation": "ATPase:ATP + 3 H+ → F1Fo ATP synthase + ATP + 3 H+", "inputs": [ { "node_id": "R-HSA-164835", "reactome_id": "R-HSA-164835", "label": "ATPase:ATP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-163953", "reactome_id": "R-ALL-163953", "label": "H+", "role": "input", "stoichiometry": 3, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-HSA-74186", "reactome_id": "R-HSA-74186", "label": "F1Fo ATP synthase", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 3, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "proton transmembrane transporter activity of ATPase:ATP [mitochondrial inner membrane]", "activity_id": "164850", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:4517936", "title": "A new concept for energy coupling in oxidative phosphorylation based on a", "journal": "Proc Natl Acad Sci U S A", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4517936/" } ], "x": 5391.333333333333, "y": 936.5, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-164840", "label": "ADP and Pi bind to ATPase", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "chemiosmosis" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:57722" ], "source_url": "https://reactome.org/content/detail/R-HSA-164840", "summary": "ADP and Pi bind to ATPase is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects ADP, Pi, F1Fo ATP synthase to ATPase:ADP:Pi in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:57722.", "equation": "ADP + Pi + F1Fo ATP synthase → ATPase:ADP:Pi", "inputs": [ { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-74186", "reactome_id": "R-HSA-74186", "label": "F1Fo ATP synthase", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-HSA-164838", "reactome_id": "R-HSA-164838", "label": "ATPase:ADP:Pi", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial inner membrane", "entity_kind": "Complex", "currency": false } ], "catalysts": [], "citations": [ { "id": "PMID:4517936", "title": "A new concept for energy coupling in oxidative phosphorylation based on a", "journal": "Proc Natl Acad Sci U S A", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4517936/" } ], "x": 5948.666666666667, "y": 936.5, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-165026", "label": "OCTN2 / SLC22A5 transports CAR from extracellular space to cytosol", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:72091" ], "source_url": "https://reactome.org/content/detail/R-HSA-165026", "summary": "OCTN2 / SLC22A5 transports CAR from extracellular space to cytosol is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects CAR, Na+ to CAR, Na+ in plasma membrane and cross-references RHEA:72091.", "equation": "CAR + Na+ → CAR + Na+", "inputs": [ { "node_id": "R-ALL-164988", "reactome_id": "R-ALL-164988", "label": "CAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-74113", "reactome_id": "R-ALL-74113", "label": "Na+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29101", "formula": "Na", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-30235", "reactome_id": "R-ALL-30235", "label": "CAR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-83910", "reactome_id": "R-ALL-83910", "label": "Na+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29101", "formula": "Na", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carnitine transmembrane transporter activity of SLC22A5 [plasma membrane]", "activity_id": "165001", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:9706223", "title": "Genetic disorders of carnitine metabolism and their nutritional management.", "journal": "Annu Rev Nutr", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9706223/" }, { "id": "PMID:26828774", "title": "Carnitine transport and fatty acid oxidation", "journal": "Biochim. Biophys. Acta", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/26828774/" }, { "id": "PMID:10559218", "title": "Mutations in novel cation transporter (OCTN2), an organic cation/carnitine transporter,with differential effects on the organic cation transport function and the carnitine transport function", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10559218/" }, { "id": "PMID:9685390", "title": "Molecular and functional identification of sodium ion-dependent, high-affinity human carnitine transporter OCTN2", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9685390/" } ], "x": 311.33333333333337, "y": 2076.8333333333335, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-1655453", "label": "FMO1:FAD oxidizes HTAU to TAU", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:69820" ], "source_url": "https://reactome.org/content/detail/R-HSA-1655453", "summary": "FMO1:FAD oxidizes HTAU to TAU is a Reactome release 97 curated reaction event. The source record connects HTAU, H+, NADPH to TAU, H2O, NADP+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:69820.", "equation": "HTAU + H+ + NADPH + O2 → TAU + H2O + NADP+", "inputs": [ { "node_id": "R-ALL-1655445", "reactome_id": "R-ALL-1655445", "label": "HTAU", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57853", "formula": "C2H7NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-159420", "reactome_id": "R-ALL-159420", "label": "TAU", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:507393", "formula": "C2H7NO3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "hypotaurine monooxygenase activity of FMO1:FAD [endoplasmic reticulum membrane]", "activity_id": "1655454", "ec_numbers": [ "1.14.13.8" ], "accessions": null } ], "citations": [ { "id": "PMID:32156684", "title": "Flavin-Containing Monooxygenase 1 Catalyzes the Production of Taurine from Hypotaurine", "journal": "Drug Metab Dispos", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/32156684/" } ], "x": 1251.3333333333333, "y": 2841.222222222222, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1678660", "label": "HGSNAT oligomer acetylates Heparan sulfate chain(3)", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "lysosomal membrane · cytosol · lysosomal lumen", "compartment_ids": [ "GO:0005765", "GO:0005829", "GO:0043202" ], "rhea_ids": [ "RHEA:15126" ], "source_url": "https://reactome.org/content/detail/R-HSA-1678660", "summary": "HGSNAT oligomer acetylates Heparan sulfate chain(3) is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, GlcN-IdoA2S-GlcNS3S- group to CoA-SH, GlcNAc-IdoA2S-GlcNS3S- group, H+ in lysosomal membrane · cytosol · lysosomal lumen and cross-references RHEA:15126.", "equation": "Ac-CoA + GlcN-IdoA2S-GlcNS3S- group → CoA-SH + GlcNAc-IdoA2S-GlcNS3S- group + H+", "inputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2090040", "reactome_id": "R-ALL-2090040", "label": "GlcN-IdoA2S-GlcNS3S- group", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:63806", "formula": "C41H67N2O43S3", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-2090069", "reactome_id": "R-ALL-2090069", "label": "GlcNAc-IdoA2S-GlcNS3S- group", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:63807", "formula": "C43H69N2O44S3", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-428040", "reactome_id": "R-ALL-428040", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "heparan-alpha-glucosaminide N-acetyltransferase activity of HGSNAT oligomer [lysosomal membrane]", "activity_id": "1678732", "ec_numbers": [ "2.3.1.78" ], "accessions": null } ], "citations": [ { "id": "PMID:20650889", "title": "Analysis of the biogenesis of heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase provides insights into the mechanism underlying its complete deficiency in mucopolysaccharidosis IIIC", "journal": "J Biol Chem", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20650889/" }, { "id": "PMID:16960811", "title": "Identification of the gene encoding the enzyme deficient in mucopolysaccharidosis IIIC (Sanfilippo disease type C)", "journal": "Am J Hum Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16960811/" }, { "id": "PMID:17033958", "title": "Mutations in TMEM76* cause mucopolysaccharidosis IIIC (Sanfilippo C syndrome)", "journal": "Am J Hum Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17033958/" } ], "x": 2938.666666666667, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-169270", "label": "ETFDH oxidises ETF (reduced) to ETF, reduces CoQ to CoQH2", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:24053" ], "source_url": "https://reactome.org/content/detail/R-HSA-169270", "summary": "ETFDH oxidises ETF (reduced) to ETF, reduces CoQ to CoQH2 is a Reactome release 97 curated reaction event. The source record connects ETF:FADH2, CoQ to ETF:FAD, H+, CoQH2 in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:24053.", "equation": "ETF:FADH2 + CoQ → ETF:FAD + H+ + CoQH2", "inputs": [ { "node_id": "R-HSA-169268", "reactome_id": "R-HSA-169268", "label": "ETF:FADH2", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-73663", "reactome_id": "R-ALL-73663", "label": "CoQ", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46245", "formula": "C59H90O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-HSA-169267", "reactome_id": "R-HSA-169267", "label": "ETF:FAD", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-2162198", "reactome_id": "R-ALL-2162198", "label": "CoQH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:64183", "formula": "C59H92O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "electron-transferring-flavoprotein dehydrogenase activity of ETFDH [mitochondrial inner membrane]", "activity_id": "169271", "ec_numbers": [ "1.5.5.1" ], "accessions": null } ], "citations": [ { "id": "PMID:1327877", "title": "Saturation kinetics of coenzyme Q in NADH and succinate oxidation in beef heart mitochondria", "journal": "FEBS Lett.", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1327877/" }, { "id": "PMID:4288886", "title": "Studies on the mechanisms of oxidative phosphorylation. IX. Effect of cytochrome c on energy-linked processes", "journal": "J. Biol. Chem.", "year": 1966, "url": "https://pubmed.ncbi.nlm.nih.gov/4288886/" } ], "x": 4451.333333333333, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-173597", "label": "UDP-glucose is oxidised to UDP-glucuronate", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23597" ], "source_url": "https://reactome.org/content/detail/R-HSA-173597", "summary": "UDP-glucose is oxidised to UDP-glucuronate is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, NAD+, H2O to UDP-GlcA, NADH, H+ in cytosol and cross-references RHEA:23597.", "equation": "UDP-Glc + 2 NAD+ + H2O → UDP-GlcA + 2 NADH + 3 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-158601", "reactome_id": "R-ALL-158601", "label": "UDP-GlcA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58052", "formula": "C15H19N2O18P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 3, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "UDP-glucose 6-dehydrogenase activity of 6xUGDH [cytosol]", "activity_id": "173601", "ec_numbers": [ "1.1.1.22" ], "accessions": null } ], "citations": [ { "id": "PMID:15044486", "title": "Characterization of human UDP-glucose dehydrogenase. CYS-276 is required for the second of two successive oxidations", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15044486/" } ], "x": 7461.333333333333, "y": 3094.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-174367", "label": "GCL ligates L-Glu to L-Cys", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13286" ], "source_url": "https://reactome.org/content/detail/R-HSA-174367", "summary": "GCL ligates L-Glu to L-Cys is a Reactome release 97 curated reaction event. The source record connects L-Glu, L-Cys, ATP to gGluCys, ADP, Pi in cytosol and cross-references RHEA:13286.", "equation": "L-Glu + L-Cys + ATP → gGluCys + ADP + Pi", "inputs": [ { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111707", "reactome_id": "R-ALL-111707", "label": "L-Cys", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:35235", "formula": "C3H7NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-174393", "reactome_id": "R-ALL-174393", "label": "gGluCys", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58173", "formula": "C8H13N2O5S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glutamate-cysteine ligase activity of GCL [cytosol]", "activity_id": "174398", "ec_numbers": [ "6.3.2.2" ], "accessions": null } ], "citations": [ { "id": "PMID:7826375", "title": "Cloning and sequencing of the cDNA for the light subunit of human liver gamma-glutamylcysteine synthetase and relative mRNA levels for heavy and light subunits in human normal tissues", "journal": "Biochem Biophys Res Commun", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7826375/" }, { "id": "PMID:9675072", "title": "Expression and purification of human gamma-glutamylcysteine synthetase", "journal": "Protein Expr Purif", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9675072/" } ], "x": 590, "y": 3479.8888888888887, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-174389", "label": "PAPSS1,2 transfer PO4(2-) group from ATP to APS to form PAPS", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24153" ], "source_url": "https://reactome.org/content/detail/R-HSA-174389", "summary": "PAPSS1,2 transfer PO4(2-) group from ATP to APS to form PAPS is a Reactome release 97 curated reaction event. The source record connects ATP, APS to ADP, PAPS, H+ in cytosol and cross-references RHEA:24153.", "equation": "ATP + APS → ADP + PAPS + H+", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-174370", "reactome_id": "R-ALL-174370", "label": "APS", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58243", "formula": "C10H12N5O10PS", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-158471", "reactome_id": "R-ALL-158471", "label": "PAPS", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58339", "formula": "C10H11N5O13P2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "adenylylsulfate kinase activity of PAPSS1,2 [cytosol]", "activity_id": "174366", "ec_numbers": [ "2.7.1.25" ], "accessions": null } ], "citations": [ { "id": "PMID:9771708", "title": "Mutations in orthologous genes in human spondyloepimetaphyseal dysplasia and the brachymorphic mouse", "journal": "Nat Genet", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9771708/" }, { "id": "PMID:9576487", "title": "Sulfation in high endothelial venules: cloning and expression of the human PAPS synthetase", "journal": "FASEB J", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9576487/" }, { "id": "PMID:9668121", "title": "Molecular cloning, expression, and characterization of human bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase and its functional domains", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9668121/" } ], "x": 2660, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-174391", "label": "MAT1A multimers transfer Ado from ATP to L-Met", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "phase-two", "sulfur-amino-acids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:21081" ], "source_url": "https://reactome.org/content/detail/R-HSA-174391", "summary": "MAT1A multimers transfer Ado from ATP to L-Met is a Reactome release 97 curated reaction event. The source record connects ATP, L-Met, H2O to AdoMet, Pi, PPi in cytosol and cross-references RHEA:21081.", "equation": "ATP + L-Met + H2O → AdoMet + Pi + PPi", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-174390", "reactome_id": "R-ALL-174390", "label": "L-Met", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57844", "formula": "C5H11NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-71284", "reactome_id": "R-ALL-71284", "label": "AdoMet", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59789", "formula": "C15H23N6O5S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "methionine adenosyltransferase activity of MAT1A multimers [cytosol]", "activity_id": "174380", "ec_numbers": [ "2.5.1.6" ], "accessions": null } ], "citations": [ { "id": "PMID:12163696", "title": "Regulation of mammalian liver methionine adenosyltransferase", "journal": "J Nutr", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12163696/" }, { "id": "PMID:9175157", "title": "S-adenosylmethionine synthesis: molecular mechanisms and clinical implications", "journal": "Pharmacol Ther", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9175157/" } ], "x": 1251.3333333333333, "y": 2917.6666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-174392", "label": "PAPSS1,2 transfer SO4(2-) group to ATP to form APS", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18134" ], "source_url": "https://reactome.org/content/detail/R-HSA-174392", "summary": "PAPSS1,2 transfer SO4(2-) group to ATP to form APS is a Reactome release 97 curated reaction event. The source record connects ATP, SO4(2-), H+ to APS, PPi in cytosol and cross-references RHEA:18134.", "equation": "ATP + SO4(2-) + H+ → APS + PPi", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-174375", "reactome_id": "R-ALL-174375", "label": "SO4(2-)", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16189", "formula": "O4S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-174370", "reactome_id": "R-ALL-174370", "label": "APS", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58243", "formula": "C10H12N5O10PS", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "sulfate adenylyltransferase (ATP) activity of PAPSS1,2 [cytosol]", "activity_id": "174381", "ec_numbers": [ "2.7.7.4" ], "accessions": null } ], "citations": [ { "id": "PMID:9576487", "title": "Sulfation in high endothelial venules: cloning and expression of the human PAPS synthetase", "journal": "FASEB J", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9576487/" }, { "id": "PMID:9771708", "title": "Mutations in orthologous genes in human spondyloepimetaphyseal dysplasia and the brachymorphic mouse", "journal": "Nat Genet", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9771708/" }, { "id": "PMID:9668121", "title": "Molecular cloning, expression, and characterization of human bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase and its functional domains", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9668121/" } ], "x": 2938.666666666667, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-174394", "label": "GSS:Mg2+ dimer synthesizes GSH", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13558" ], "source_url": "https://reactome.org/content/detail/R-HSA-174394", "summary": "GSS:Mg2+ dimer synthesizes GSH is a Reactome release 97 curated reaction event. The source record connects gGluCys, Gly, ATP to GSH, ADP, Pi in cytosol and cross-references RHEA:13558.", "equation": "gGluCys + Gly + ATP → GSH + ADP + Pi", "inputs": [ { "node_id": "R-ALL-174393", "reactome_id": "R-ALL-174393", "label": "gGluCys", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58173", "formula": "C8H13N2O5S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29424", "reactome_id": "R-ALL-29424", "label": "Gly", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29450", "reactome_id": "R-ALL-29450", "label": "GSH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57925", "formula": "C10H16N3O6S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glutathione synthase activity of GSS:Mg2+ dimer [cytosol]", "activity_id": "174399", "ec_numbers": [ "6.3.2.3" ], "accessions": null } ], "citations": [ { "id": "PMID:7646467", "title": "Sequencing and expression of a cDNA for human glutathione synthetase", "journal": "Biochem J", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7646467/" }, { "id": "PMID:10369661", "title": "Molecular basis of glutathione synthetase deficiency and a rare gene permutation event", "journal": "EMBO J.", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10369661/" } ], "x": 868.6666666666667, "y": 3479.8888888888887, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-174401", "label": "AHCY:NAD+ tetramer hydrolyses AdoHcy", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-two", "sulfur-amino-acids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:21709" ], "source_url": "https://reactome.org/content/detail/R-HSA-174401", "summary": "AHCY:NAD+ tetramer hydrolyses AdoHcy is a Reactome release 97 curated reaction event. The source record connects AdoHcy, H2O to HCYS, Ade-Rib in cytosol and cross-references RHEA:21709.", "equation": "AdoHcy + H2O → HCYS + Ade-Rib", "inputs": [ { "node_id": "R-ALL-71285", "reactome_id": "R-ALL-71285", "label": "AdoHcy", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57856", "formula": "C14H20N6O5S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-174369", "reactome_id": "R-ALL-174369", "label": "HCYS", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58199", "formula": "C4H9NO2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-83909", "reactome_id": "R-ALL-83909", "label": "Ade-Rib", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16335", "formula": "C10H13N5O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "adenosylhomocysteinase activity of AHCY:NAD+ tetramer [cytosol]", "activity_id": "174397", "ec_numbers": [ "3.13.2.1" ], "accessions": null } ], "citations": [ { "id": "PMID:9586999", "title": "Structure determination of selenomethionyl S-adenosylhomocysteine hydrolase using data at a single wavelength", "journal": "Nat. Struct. Biol.", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9586999/" }, { "id": "PMID:12590576", "title": "Catalytic strategy of S-adenosyl-L-homocysteine hydrolase: transition-state stabilization and the avoidance of abortive reactions", "journal": "Biochemistry", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12590576/" } ], "x": 7740, "y": 3094.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855153", "label": "I(1,4,5)P3 is phosphorylated to I(1,3,4,5)P4 by ITPKA/B/C in the cytosol", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11021" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855153", "summary": "I(1,4,5)P3 is phosphorylated to I(1,3,4,5)P4 by ITPKA/B/C in the cytosol is a Reactome release 97 curated reaction event. The source record connects I(1,4,5)P3, ATP to I(1,3,4,5)P4, ADP, H+ in cytosol and cross-references RHEA:11021.", "equation": "I(1,4,5)P3 + ATP → I(1,3,4,5)P4 + ADP + H+", "inputs": [ { "node_id": "R-ALL-114520", "reactome_id": "R-ALL-114520", "label": "I(1,4,5)P3", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:203600", "formula": "C6H9O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-994170", "reactome_id": "R-ALL-994170", "label": "I(1,3,4,5)P4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57895", "formula": "C6H8O18P4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "inositol-1,4,5-trisphosphate 3-kinase activity of active ITPKA/B/C [cytosol]", "activity_id": "2023862", "ec_numbers": [ "2.7.1.127" ], "accessions": null } ], "citations": [ { "id": "PMID:12747803", "title": "The three isoenzymes of human inositol-1,4,5-trisphosphate 3-kinase show specific intracellular localization but comparable Ca2+ responses on transfection in COS-7 cells", "journal": "Biochem J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12747803/" } ], "x": 7461.333333333333, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855157", "label": "1-PP-IP5 is phosphorylated to 1,5-(PP)2-IP4 by IP6K1/2 in the nucleus", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "nucleoplasm", "compartment_ids": [ "GO:0005654" ], "rhea_ids": [ "RHEA:37468" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855157", "summary": "1-PP-IP5 is phosphorylated to 1,5-(PP)2-IP4 by IP6K1/2 in the nucleus is a Reactome release 97 curated reaction event. The source record connects 1-PP-IP5, ATP, H+ to 1,5-(PP)2-IP4, ADP in nucleoplasm and cross-references RHEA:37468.", "equation": "1-PP-IP5 + ATP + H+ → 1,5-(PP)2-IP4 + ADP", "inputs": [ { "node_id": "R-ALL-2023932", "reactome_id": "R-ALL-2023932", "label": "1-PP-IP5", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:74946", "formula": "C6H6O27P7", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29358", "reactome_id": "R-ALL-29358", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9668967", "reactome_id": "R-ALL-9668967", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2023931", "reactome_id": "R-ALL-2023931", "label": "1,5-(PP)2-IP4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:77983", "formula": "C6H7O30P8", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113582", "reactome_id": "R-ALL-113582", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "diphosphoinositol pentakisphosphate kinase activity of IP6K1/2 [nucleoplasm]", "activity_id": "2023919", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:11502751", "title": "Identification and characterization of a novel inositol hexakisphosphate kinase", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11502751/" }, { "id": "PMID:17412958", "title": "A conserved family of enzymes that phosphorylate inositol hexakisphosphate", "journal": "Science", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17412958/" } ], "x": 7740, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855169", "label": "I(3,4,6)P3 is phosphorylated to I(1,3,4,6)P4 by ITPK1 in the cytosol", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:70288" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855169", "summary": "I(3,4,6)P3 is phosphorylated to I(1,3,4,6)P4 by ITPK1 in the cytosol is a Reactome release 97 curated reaction event. The source record connects I(3,4,6)P3, ATP to I(1,3,4,6)P4, ADP, H+ in cytosol and cross-references RHEA:70288.", "equation": "I(3,4,6)P3 + ATP → I(1,3,4,6)P4 + ADP + H+", "inputs": [ { "node_id": "R-ALL-1629741", "reactome_id": "R-ALL-1629741", "label": "I(3,4,6)P3", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:189099", "formula": "C6H9O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-994146", "reactome_id": "R-ALL-994146", "label": "I(1,3,4,6)P4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57660", "formula": "C6H8O18P4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "inositol-3,4,6-trisphosphate 1-kinase activity of ITPK1:Mg2+ [cytosol]", "activity_id": "2023895", "ec_numbers": [ "2.7.1" ], "accessions": null } ], "citations": [ { "id": "PMID:11909533", "title": "Regulation of Ins(3,4,5,6)P(4) signaling by a reversible kinase/phosphatase", "journal": "Curr Biol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11909533/" } ], "x": 8018.666666666667, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855172", "label": "I(1,3,4)P3 is phosphorylated to I(1,3,4,5)P4 by ITPK1 in the cytosol", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13254" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855172", "summary": "I(1,3,4)P3 is phosphorylated to I(1,3,4,5)P4 by ITPK1 in the cytosol is a Reactome release 97 curated reaction event. The source record connects I(1,3,4)P3, ATP to I(1,3,4,5)P4, ADP, H+ in cytosol and cross-references RHEA:13254.", "equation": "I(1,3,4)P3 + ATP → I(1,3,4,5)P4 + ADP + H+", "inputs": [ { "node_id": "R-ALL-994123", "reactome_id": "R-ALL-994123", "label": "I(1,3,4)P3", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58414", "formula": "C6H9O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-994170", "reactome_id": "R-ALL-994170", "label": "I(1,3,4,5)P4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57895", "formula": "C6H8O18P4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "inositol-1,3,4-trisphosphate 5-kinase activity of ITPK1:Mg2+ [cytosol]", "activity_id": "2023874", "ec_numbers": [ "2.7.1.159" ], "accessions": null } ], "citations": [ { "id": "PMID:8662638", "title": "Isolation of inositol 1,3,4-trisphosphate 5/6-kinase, cDNA cloning and expression of the recombinant enzyme", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8662638/" } ], "x": 7461.333333333333, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855176", "label": "I(1,3,4,5,6)P5 is phosphorylated to IP6 by IPPK (IP5-2K) in the nucleus", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "nucleoplasm", "compartment_ids": [ "GO:0005654" ], "rhea_ids": [ "RHEA:20314" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855176", "summary": "I(1,3,4,5,6)P5 is phosphorylated to IP6 by IPPK (IP5-2K) in the nucleus is a Reactome release 97 curated reaction event. The source record connects I(1,3,4,5,6)P5, ATP to IP6, ADP, H+ in nucleoplasm and cross-references RHEA:20314.", "equation": "I(1,3,4,5,6)P5 + ATP → IP6 + ADP + H+", "inputs": [ { "node_id": "R-ALL-2023901", "reactome_id": "R-ALL-2023901", "label": "I(1,3,4,5,6)P5", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57733", "formula": "C6H7O21P5", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29358", "reactome_id": "R-ALL-29358", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2023929", "reactome_id": "R-ALL-2023929", "label": "IP6", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58130", "formula": "C6H6O24P6", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113582", "reactome_id": "R-ALL-113582", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9668967", "reactome_id": "R-ALL-9668967", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "inositol-1,3,4,5,6-pentakisphosphate 2-kinase activity of IPPK [nucleoplasm]", "activity_id": "2023933", "ec_numbers": [ "2.7.1.158" ], "accessions": null } ], "citations": [ { "id": "PMID:12084730", "title": "The synthesis of inositol hexakisphosphate. Characterization of human inositol 1,3,4,5,6-pentakisphosphate 2-kinase", "journal": "J Biol Chem", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12084730/" }, { "id": "PMID:17705785", "title": "Intracellular localization of human Ins(1,3,4,5,6)P5 2-kinase", "journal": "Biochem J", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17705785/" }, { "id": "PMID:17702752", "title": "Purification, sequencing, and molecular identification of a mammalian PP-InsP5 kinase that is activated when cells are exposed to hyperosmotic stress", "journal": "J Biol Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17702752/" } ], "x": 7740, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855177", "label": "PI(4,5)P2 is hydrolysed to I(1,4,5)P3 and DAG by cytosolic PLC[2] at the plasma membrane", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "cytosol · plasma membrane", "compartment_ids": [ "GO:0005829", "GO:0005886" ], "rhea_ids": [ "RHEA:33180", "RHEA:33179" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855177", "summary": "PI(4,5)P2 is hydrolysed to I(1,4,5)P3 and DAG by cytosolic PLC[2] at the plasma membrane is a Reactome release 97 curated reaction event. The source record connects PI(4,5)P2, H2O to DAG, I(1,4,5)P3, H+ in cytosol · plasma membrane and cross-references RHEA:33180, RHEA:33179.", "equation": "PI(4,5)P2 + H2O → DAG + I(1,4,5)P3 + H+", "inputs": [ { "node_id": "R-ALL-179856", "reactome_id": "R-ALL-179856", "label": "PI(4,5)P2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58456", "formula": "C11H14O19P3R2", "compartment": "plasma membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-114519", "reactome_id": "R-ALL-114519", "label": "DAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "plasma membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-114520", "reactome_id": "R-ALL-114520", "label": "I(1,4,5)P3", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:203600", "formula": "C6H9O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphatidylinositol-4,5-bisphosphate phospholipase C activity of PLCbz [cytosol]", "activity_id": "2023847", "ec_numbers": [ "3.1.4.11" ], "accessions": null } ], "citations": [ { "id": "PMID:11118617", "title": "Cloning and characterization of the human phosphoinositide-specific phospholipase C-beta 1 (PLC beta 1)", "journal": "Biochim Biophys Acta", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/11118617/" }, { "id": "PMID:1333955", "title": "Identification, purification and characterization of a novel phosphatidylinositol-specific phospholipase C, a third member of the beta subfamily", "journal": "Eur J Biochem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1333955/" }, { "id": "PMID:15790568", "title": "The role of EF-hand domains and C2 domain in regulation of enzymatic activity of phospholipase Czeta", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15790568/" }, { "id": "PMID:8454637", "title": "Cloning, sequencing, purification, and Gq-dependent activation of phospholipase C-beta 3", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8454637/" }, { "id": "PMID:8530101", "title": "cDNA sequence and gene locus of the human retinal phosphoinositide-specific phospholipase-C beta 4 (PLCB4)", "journal": "Genomics", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/8530101/" }, { "id": "PMID:15579586", "title": "Phospholipase Czeta causes Ca2+ oscillations and parthenogenetic activation of human oocytes", "journal": "Reproduction", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15579586/" } ], "x": 8018.666666666667, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855185", "label": "I(1,4,5,6)P4 is phosphorylated to I(1,3,4,5,6)P5 by IPMK in the nucleus", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "nucleoplasm", "compartment_ids": [ "GO:0005654" ], "rhea_ids": [ "RHEA:11857" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855185", "summary": "I(1,4,5,6)P4 is phosphorylated to I(1,3,4,5,6)P5 by IPMK in the nucleus is a Reactome release 97 curated reaction event. The source record connects I(1,4,5,6)P4, ATP to I(1,3,4,5,6)P5, ADP, H+ in nucleoplasm and cross-references RHEA:11857.", "equation": "I(1,4,5,6)P4 + ATP → I(1,3,4,5,6)P5 + ADP + H+", "inputs": [ { "node_id": "R-ALL-2023935", "reactome_id": "R-ALL-2023935", "label": "I(1,4,5,6)P4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57627", "formula": "C6H8O18P4", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29358", "reactome_id": "R-ALL-29358", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2023901", "reactome_id": "R-ALL-2023901", "label": "I(1,3,4,5,6)P5", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57733", "formula": "C6H7O21P5", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113582", "reactome_id": "R-ALL-113582", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9668967", "reactome_id": "R-ALL-9668967", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "inositol-1,4,5,6-tetrakisphosphate 3-kinase activity of IPMK [nucleoplasm]", "activity_id": "2023945", "ec_numbers": [ "2.7.1.151" ], "accessions": null } ], "citations": [ { "id": "PMID:12027805", "title": "The human homologue of yeast ArgRIII protein is an inositol phosphate multikinase with predominantly nuclear localization", "journal": "Biochem J", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12027805/" }, { "id": "PMID:16293229", "title": "Inositol polyphosphate multikinase regulates inositol 1,4,5,6-tetrakisphosphate", "journal": "Biochem Biophys Res Commun", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16293229/" } ], "x": 7461.333333333333, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855197", "label": "I(1,3,4)P3 is phosphorylated to I(1,3,4,6)P4 by ITPK1 in the cytosol", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:20941" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855197", "summary": "I(1,3,4)P3 is phosphorylated to I(1,3,4,6)P4 by ITPK1 in the cytosol is a Reactome release 97 curated reaction event. The source record connects I(1,3,4)P3, ATP to I(1,3,4,6)P4, ADP, H+ in cytosol and cross-references RHEA:20941.", "equation": "I(1,3,4)P3 + ATP → I(1,3,4,6)P4 + ADP + H+", "inputs": [ { "node_id": "R-ALL-994123", "reactome_id": "R-ALL-994123", "label": "I(1,3,4)P3", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58414", "formula": "C6H9O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-994146", "reactome_id": "R-ALL-994146", "label": "I(1,3,4,6)P4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57660", "formula": "C6H8O18P4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "inositol-1,3,4-trisphosphate 6-kinase activity of ITPK1:Mg2+ [cytosol]", "activity_id": "2023853", "ec_numbers": [ "2.7.1.159" ], "accessions": null } ], "citations": [ { "id": "PMID:8662638", "title": "Isolation of inositol 1,3,4-trisphosphate 5/6-kinase, cDNA cloning and expression of the recombinant enzyme", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8662638/" }, { "id": "PMID:11042108", "title": "Multitasking in signal transduction by a promiscuous human Ins(3,4,5,6)P(4) 1-kinase/Ins(1,3,4)P(3) 5/6-kinase", "journal": "Biochem J", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/11042108/" } ], "x": 7740, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855214", "label": "PL(C)D4:3xCa2+ hydrolse PI(4,5)P2 to I(1,4,5)P3 and DAG at the ER membrane", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:33179", "RHEA:33180" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855214", "summary": "PL(C)D4:3xCa2+ hydrolse PI(4,5)P2 to I(1,4,5)P3 and DAG at the ER membrane is a Reactome release 97 curated reaction event. The source record connects PI(4,5)P2, H2O to DAG, I(1,4,5)P3, H+ in endoplasmic reticulum membrane · cytosol and cross-references RHEA:33179, RHEA:33180.", "equation": "PI(4,5)P2 + H2O → DAG + I(1,4,5)P3 + H+", "inputs": [ { "node_id": "R-ALL-2023856", "reactome_id": "R-ALL-2023856", "label": "PI(4,5)P2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58456", "formula": "C11H14O19P3R2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-1500596", "reactome_id": "R-ALL-1500596", "label": "DAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-114520", "reactome_id": "R-ALL-114520", "label": "I(1,4,5)P3", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:203600", "formula": "C6H9O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphatidylinositol-4,5-bisphosphate phospholipase C activity of PL(C)D4:3xCa2+ [endoplasmic reticulum membrane]", "activity_id": "2023871", "ec_numbers": [ "3.1.4.11" ], "accessions": null } ], "citations": [ { "id": "PMID:15140260", "title": "Phospholipase C delta-4 overexpression upregulates ErbB1/2 expression, Erk signaling pathway, and proliferation in MCF-7 cells", "journal": "Mol Cancer", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15140260/" }, { "id": "PMID:15037625", "title": "The pleckstrin homology domain of phosphoinositide-specific phospholipase Cdelta4 is not a critical determinant of the membrane localization of the enzyme", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15037625/" } ], "x": 8018.666666666667, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855221", "label": "PI(4,5)P2 is hydrolysed to I(1,4,5)P3 and DAG by tethered PLC[1] at the plasma membrane", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "plasma membrane · cytosol", "compartment_ids": [ "GO:0005886", "GO:0005829" ], "rhea_ids": [ "RHEA:33180", "RHEA:33179" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855221", "summary": "PI(4,5)P2 is hydrolysed to I(1,4,5)P3 and DAG by tethered PLC[1] at the plasma membrane is a Reactome release 97 curated reaction event. The source record connects PI(4,5)P2, H2O to DAG, I(1,4,5)P3, H+ in plasma membrane · cytosol and cross-references RHEA:33180, RHEA:33179.", "equation": "PI(4,5)P2 + H2O → DAG + I(1,4,5)P3 + H+", "inputs": [ { "node_id": "R-ALL-179856", "reactome_id": "R-ALL-179856", "label": "PI(4,5)P2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58456", "formula": "C11H14O19P3R2", "compartment": "plasma membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-114519", "reactome_id": "R-ALL-114519", "label": "DAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "plasma membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-114520", "reactome_id": "R-ALL-114520", "label": "I(1,4,5)P3", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:203600", "formula": "C6H9O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphatidylinositol-4,5-bisphosphate phospholipase C activity of PLCdegh [plasma membrane]", "activity_id": "2023860", "ec_numbers": [ "3.1.4.11" ], "accessions": null } ], "citations": [ { "id": "PMID:7890667", "title": "Cloning and identification of amino acid residues of human phospholipase C delta 1 essential for catalysis", "journal": "J Biol Chem", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7890667/" }, { "id": "PMID:11022047", "title": "A novel bifunctional phospholipase c that is regulated by Galpha 12 and stimulates the Ras/mitogen-activated protein kinase pathway", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11022047/" }, { "id": "PMID:2841328", "title": "Purification and characterization of membrane-bound phospholipase C specific for phosphoinositides from human platelets", "journal": "J Biol Chem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/2841328/" }, { "id": "PMID:9360711", "title": "Expression, purification and kinetic properties of human recombinant phospholipase C delta 3", "journal": "Acta Biochim Pol", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9360711/" }, { "id": "PMID:19179337", "title": "Phosphorylation of Nephrin Triggers Ca2+ Signaling by Recruitment and Activation of Phospholipase C-{gamma}1", "journal": "J Biol Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19179337/" }, { "id": "PMID:15702972", "title": "Molecular cloning and characterization of a novel phospholipase C, PLC-eta", "journal": "Biochem J", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15702972/" } ], "x": 7461.333333333333, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-1855228", "label": "I(1,3,4,6)P4 is phosphorylated to I(1,3,4,5,6)P5 by IPMK in the nucleus", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "inositol-phosphate" ], "compartment": "nucleoplasm", "compartment_ids": [ "GO:0005654" ], "rhea_ids": [ "RHEA:12718" ], "source_url": "https://reactome.org/content/detail/R-HSA-1855228", "summary": "I(1,3,4,6)P4 is phosphorylated to I(1,3,4,5,6)P5 by IPMK in the nucleus is a Reactome release 97 curated reaction event. The source record connects I(1,3,4,6)P4, ATP to I(1,3,4,5,6)P5, ADP, H+ in nucleoplasm and cross-references RHEA:12718.", "equation": "I(1,3,4,6)P4 + ATP → I(1,3,4,5,6)P5 + ADP + H+", "inputs": [ { "node_id": "R-ALL-2023892", "reactome_id": "R-ALL-2023892", "label": "I(1,3,4,6)P4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57660", "formula": "C6H8O18P4", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29358", "reactome_id": "R-ALL-29358", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2023901", "reactome_id": "R-ALL-2023901", "label": "I(1,3,4,5,6)P5", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57733", "formula": "C6H7O21P5", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113582", "reactome_id": "R-ALL-113582", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9668967", "reactome_id": "R-ALL-9668967", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "inositol-1,3,4,6-tetrakisphosphate 5-kinase activity of IPMK [nucleoplasm]", "activity_id": "2023903", "ec_numbers": [ "2.7.1.140" ], "accessions": null } ], "citations": [ { "id": "PMID:12223481", "title": "The human homolog of the rat inositol phosphate multikinase is an inositol 1,3,4,6-tetrakisphosphate 5-kinase", "journal": "J Biol Chem", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12223481/" }, { "id": "PMID:16293229", "title": "Inositol polyphosphate multikinase regulates inositol 1,4,5,6-tetrakisphosphate", "journal": "Biochem Biophys Res Commun", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16293229/" } ], "x": 7740, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-188467", "label": "Gly-3-P+FAD-\u003eDHAP+FADH2 (catalyzed by mitochondrial Gly-Phos dehydrogenase)", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids", "triglycerides" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:31284" ], "source_url": "https://reactome.org/content/detail/R-HSA-188467", "summary": "Gly-3-P+FAD-\u003eDHAP+FADH2 (catalyzed by mitochondrial Gly-Phos dehydrogenase) is a Reactome release 97 curated reaction event. The source record connects G3P, FAD to DHAP, FADH2 in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:31284.", "equation": "G3P + FAD → DHAP + FADH2", "inputs": [ { "node_id": "R-ALL-188458", "reactome_id": "R-ALL-188458", "label": "G3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57597", "formula": "C3H7O6P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113596", "reactome_id": "R-ALL-113596", "label": "FAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57692", "formula": "C27H30N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-188451", "reactome_id": "R-ALL-188451", "label": "DHAP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57642", "formula": "C3H5O6P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31649", "reactome_id": "R-ALL-31649", "label": "FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58307", "formula": "C27H33N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glycerol-3-phosphate dehydrogenase (quinone) activity of GPD2 [mitochondrial inner membrane]", "activity_id": "188456", "ec_numbers": [ "1.1.5.3" ], "accessions": null } ], "citations": [ { "id": "PMID:7821823", "title": "The sequence of a human mitochondrial glycerol-3-phosphate dehydrogenase-encoding cDNA", "journal": "Gene", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7821823/" }, { "id": "PMID:8687421", "title": "Molecular cloning of human mitochondrial glycerophosphate dehydrogenase gene: genomic structure, chromosomal localization, and existence of a pseudogene", "journal": "Biochem Biophys Res Commun", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8687421/" }, { "id": "PMID:8163052", "title": "Enzymatic, metabolic and secretory patterns in human islets of type 2 (non-insulin-dependent) diabetic patients", "journal": "Diabetologia", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8163052/" } ], "x": 3600, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189384", "label": "BLVRA:Zn2+, BLVRB reduce BV to BIL", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15795" ], "source_url": "https://reactome.org/content/detail/R-HSA-189384", "summary": "BLVRA:Zn2+, BLVRB reduce BV to BIL is a Reactome release 97 curated reaction event. The source record connects BV, NADPH to BIL, NADP+ in cytosol and cross-references RHEA:15795.", "equation": "BV + NADPH → BIL + NADP+", "inputs": [ { "node_id": "R-ALL-189396", "reactome_id": "R-ALL-189396", "label": "BV", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57991", "formula": "C33H32N4O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-189386", "reactome_id": "R-ALL-189386", "label": "BIL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57977", "formula": "C33H34N4O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "biliverdin reductase [NAD(P)H] activity of BLVRA:Zn2+,BLVRB [cytosol]", "activity_id": "189392", "ec_numbers": [ "1.3.1.24" ], "accessions": null } ], "citations": [ { "id": "PMID:22823425", "title": "Molecular modeling to provide insight into the substrate binding and catalytic mechanism of human biliverdin-IXα reductase", "journal": "J Phys Chem B", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22823425/" }, { "id": "PMID:10620517", "title": "Initial-rate kinetics of the flavin reductase reaction catalysed by human biliverdin-IXbeta reductase (BVR-B)", "journal": "Biochem. J.", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10620517/" } ], "x": 7078.666666666667, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189406", "label": "4 PBGs bind to form HMB", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13186" ], "source_url": "https://reactome.org/content/detail/R-HSA-189406", "summary": "4 PBGs bind to form HMB is a Reactome release 97 curated reaction event. The source record connects PBG, H2O to HMBL, NH4+ in cytosol and cross-references RHEA:13186.", "equation": "4 PBG + H2O → HMBL + 4 NH4+", "inputs": [ { "node_id": "R-ALL-189464", "reactome_id": "R-ALL-189464", "label": "PBG", "role": "input", "stoichiometry": 4, "chebi_id": "CHEBI:58126", "formula": "C10H13N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-189449", "reactome_id": "R-ALL-189449", "label": "HMBL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57845", "formula": "C40H38N4O17", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "hydroxymethylbilane synthase activity of HMBS:DIPY [cytosol]", "activity_id": "189486", "ec_numbers": [ "2.5.1.61" ], "accessions": null } ], "citations": [ { "id": "PMID:12773194", "title": "Human porphobilinogen deaminase mutations in the investigation of the mechanism of dipyrromethane cofactor assembly and tetrapyrrole formation", "journal": "Biochem Soc Trans", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12773194/" }, { "id": "PMID:7354069", "title": "Purification and properties of uroporphyrinogen I synthase from human erythrocytes. Identification of stable enzyme-substrate intermediates.", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/7354069/" } ], "x": 6521.333333333333, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189421", "label": "CPO transforms COPRO3 to PPGEN9", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "mitochondrial intermembrane space", "compartment_ids": [ "GO:0005758" ], "rhea_ids": [ "RHEA:18258" ], "source_url": "https://reactome.org/content/detail/R-HSA-189421", "summary": "CPO transforms COPRO3 to PPGEN9 is a Reactome release 97 curated reaction event. The source record connects COPRO3, O2, H+ to PPGEN9, CO2, H2O in mitochondrial intermembrane space and cross-references RHEA:18258.", "equation": "COPRO3 + O2 + H+ → PPGEN9 + 2 CO2 + H2O", "inputs": [ { "node_id": "R-ALL-189411", "reactome_id": "R-ALL-189411", "label": "COPRO3", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57309", "formula": "C36H40N4O8", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-189461", "reactome_id": "R-ALL-189461", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-163953", "reactome_id": "R-ALL-163953", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-189478", "reactome_id": "R-ALL-189478", "label": "PPGEN9", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57307", "formula": "C34H38N4O4", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-189480", "reactome_id": "R-ALL-189480", "label": "CO2", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-189422", "reactome_id": "R-ALL-189422", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "coproporphyrinogen oxidase activity of 2xCPO [mitochondrial intermembrane space]", "activity_id": "189416", "ec_numbers": [ "1.3.3.3" ], "accessions": null } ], "citations": [ { "id": "PMID:16258391", "title": "Kinetic evaluation of human cloned coproporphyrinogen oxidase using a ring isomer of the natural substrate", "journal": "Med Sci Monit", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16258391/" }, { "id": "PMID:666752", "title": "Evidence that the coproporphyrinogen oxidase activity of rat liver is situated in the intermembrane space of mitochondria", "journal": "Biochem J", "year": 1978, "url": "https://pubmed.ncbi.nlm.nih.gov/666752/" }, { "id": "PMID:16176984", "title": "Structural basis of hereditary coproporphyria", "journal": "Proc Natl Acad Sci U S A", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16176984/" }, { "id": "PMID:8159699", "title": "Molecular cloning, sequencing, and functional expression of a cDNA encoding human coproporphyrinogen oxidase", "journal": "Proc Natl Acad Sci U S A", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8159699/" } ], "x": 7078.666666666667, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189423", "label": "PPO oxidises PPGEN9 to PRIN9", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "mitochondrial intermembrane space", "compartment_ids": [ "GO:0005758" ], "rhea_ids": [ "RHEA:25577" ], "source_url": "https://reactome.org/content/detail/R-HSA-189423", "summary": "PPO oxidises PPGEN9 to PRIN9 is a Reactome release 97 curated reaction event. The source record connects PPGEN9, O2 to PRIN9, H2O2 in mitochondrial intermembrane space and cross-references RHEA:25577.", "equation": "PPGEN9 + 3 O2 → PRIN9 + 3 H2O2", "inputs": [ { "node_id": "R-ALL-189478", "reactome_id": "R-ALL-189478", "label": "PPGEN9", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57307", "formula": "C34H38N4O4", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-189461", "reactome_id": "R-ALL-189461", "label": "O2", "role": "input", "stoichiometry": 3, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-189463", "reactome_id": "R-ALL-189463", "label": "PRIN9", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57306", "formula": "C34H32N4O4", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-189481", "reactome_id": "R-ALL-189481", "label": "H2O2", "role": "output", "stoichiometry": 3, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "oxygen-dependent protoporphyrinogen oxidase activity of 2xPPOX:FAD [mitochondrial intermembrane space]", "activity_id": "189420", "ec_numbers": [ "1.3.3.4" ], "accessions": null } ], "citations": [ { "id": "PMID:8771201", "title": "Human protoporphyrinogen oxidase: expression, purification, and characterization of the cloned enzyme", "journal": "Protein Sci", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8771201/" }, { "id": "PMID:23467411", "title": "Quantitative structural insight into human variegate porphyria disease", "journal": "J Biol Chem", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23467411/" } ], "x": 6800, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189425", "label": "UROD decarboxylates URO3 to COPRO3", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19866" ], "source_url": "https://reactome.org/content/detail/R-HSA-189425", "summary": "UROD decarboxylates URO3 to COPRO3 is a Reactome release 97 curated reaction event. The source record connects URO3, H+ to COPRO3, CO2 in cytosol and cross-references RHEA:19866.", "equation": "URO3 + 4 H+ → COPRO3 + 4 CO2", "inputs": [ { "node_id": "R-ALL-189407", "reactome_id": "R-ALL-189407", "label": "URO3", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57308", "formula": "C40H36N4O16", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 4, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-189455", "reactome_id": "R-ALL-189455", "label": "COPRO3", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57309", "formula": "C36H40N4O8", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carbon-carbon lyase activity of 2xUROD [cytosol]", "activity_id": "189412", "ec_numbers": [ "4.1" ], "accessions": null } ], "citations": [ { "id": "PMID:6822570", "title": "Purification and properties of uroporphyrinogen decarboxylase from human erythrocytes. A single enzyme catalyzing the four sequential decarboxylations of uroporphyrinogens I and III.", "journal": "J Biol Chem", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/6822570/" }, { "id": "PMID:8644733", "title": "Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria", "journal": "Am J Hum Genet", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8644733/" } ], "x": 7078.666666666667, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189439", "label": "ALAD condenses 2 dALAs to form PBG", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24065" ], "source_url": "https://reactome.org/content/detail/R-HSA-189439", "summary": "ALAD condenses 2 dALAs to form PBG is a Reactome release 97 curated reaction event. The source record connects dALA to PBG, H2O, H+ in cytosol and cross-references RHEA:24065.", "equation": "2 dALA → PBG + 2 H2O + H+", "inputs": [ { "node_id": "R-ALL-189489", "reactome_id": "R-ALL-189489", "label": "dALA", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:356416", "formula": "C5H9NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-189464", "reactome_id": "R-ALL-189464", "label": "PBG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58126", "formula": "C10H13N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "porphobilinogen synthase activity of 8x(ALAD:Zn2+) [cytosol]", "activity_id": "189415", "ec_numbers": [ "4.2.1.24" ], "accessions": null } ], "citations": [ { "id": "PMID:11032841", "title": "Mechanistic implications of mutations to the active site lysine of porphobilinogen synthase", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11032841/" }, { "id": "PMID:10706561", "title": "Novel molecular defects of the delta-aminolevulinate dehydratase gene in a patient with inherited acute hepatic porphyria", "journal": "Hepatology", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10706561/" } ], "x": 6521.333333333333, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189442", "label": "ALAS condenses SUCC-CoA and Gly to form dALA", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:12922" ], "source_url": "https://reactome.org/content/detail/R-HSA-189442", "summary": "ALAS condenses SUCC-CoA and Gly to form dALA is a Reactome release 97 curated reaction event. The source record connects SUCC-CoA, Gly, H+ to dALA, CoA-SH, CO2 in mitochondrial matrix and cross-references RHEA:12922.", "equation": "SUCC-CoA + Gly + H+ → dALA + CoA-SH + CO2", "inputs": [ { "node_id": "R-ALL-70958", "reactome_id": "R-ALL-70958", "label": "SUCC-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57292", "formula": "C25H35N7O19P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159549", "reactome_id": "R-ALL-159549", "label": "Gly", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-189466", "reactome_id": "R-ALL-189466", "label": "dALA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:356416", "formula": "C5H9NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "5-aminolevulinate synthase activity of ALAS1,2 [mitochondrial matrix]", "activity_id": "189479", "ec_numbers": [ "2.3.1.37" ], "accessions": null } ], "citations": [ { "id": "PMID:1939222", "title": "Biosynthesis of delta-aminolevulinic acid and the regulation of heme formation by immature erythroid cells in man", "journal": "J Biol Chem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1939222/" }, { "id": "PMID:16121195", "title": "Crystal structure of 5-aminolevulinate synthase, the first enzyme of heme biosynthesis, and its link to XLSA in humans", "journal": "EMBO J", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16121195/" } ], "x": 6521.333333333333, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-189488", "label": "UROS transforms HMB to URO3", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18966" ], "source_url": "https://reactome.org/content/detail/R-HSA-189488", "summary": "UROS transforms HMB to URO3 is a Reactome release 97 curated reaction event. The source record connects HMBL to URO3, H2O in cytosol and cross-references RHEA:18966.", "equation": "HMBL → URO3 + H2O", "inputs": [ { "node_id": "R-ALL-189449", "reactome_id": "R-ALL-189449", "label": "HMBL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57845", "formula": "C40H38N4O17", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-189407", "reactome_id": "R-ALL-189407", "label": "URO3", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57308", "formula": "C40H36N4O16", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "uroporphyrinogen-III synthase activity of UROS [cytosol]", "activity_id": "189468", "ec_numbers": [ "4.2.1.75" ], "accessions": null } ], "citations": [ { "id": "PMID:7592565", "title": "Porphobilinogen deaminase and uroporphyrinogen III synthase: structure, molecular biology, and mechanism", "journal": "J Bioenerg Biomembr", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7592565/" }, { "id": "PMID:3805019", "title": "Purification and properties of uroporphyrinogen III synthase from human erythrocytes", "journal": "J Biol Chem", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3805019/" } ], "x": 7078.666666666667, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-190182", "label": "UROD decarboxylates URO1 to COPRO1", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:31240" ], "source_url": "https://reactome.org/content/detail/R-HSA-190182", "summary": "UROD decarboxylates URO1 to COPRO1 is a Reactome release 97 curated reaction event. The source record connects URO1, H+ to COPRO1, CO2 in cytosol and cross-references RHEA:31240.", "equation": "URO1 + 4 H+ → COPRO1 + 4 CO2", "inputs": [ { "node_id": "R-ALL-190157", "reactome_id": "R-ALL-190157", "label": "URO1", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62626", "formula": "C40H36N4O16", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 4, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-190128", "reactome_id": "R-ALL-190128", "label": "COPRO1", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62631", "formula": "C36H40N4O8", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carbon-carbon lyase activity of 2xUROD [cytosol]", "activity_id": "189412", "ec_numbers": [ "4.1" ], "accessions": null } ], "citations": [ { "id": "PMID:6822570", "title": "Purification and properties of uroporphyrinogen decarboxylase from human erythrocytes. A single enzyme catalyzing the four sequential decarboxylations of uroporphyrinogens I and III.", "journal": "J Biol Chem", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/6822570/" }, { "id": "PMID:8644733", "title": "Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria", "journal": "Am J Hum Genet", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8644733/" } ], "x": 6800, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-191323", "label": "HMGCS1 condenses Ac-CoA and ACA-CoA to form bHMG-CoA", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10189" ], "source_url": "https://reactome.org/content/detail/R-HSA-191323", "summary": "HMGCS1 condenses Ac-CoA and ACA-CoA to form bHMG-CoA is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, ACA-CoA, H2O to CoA-SH, bHMG-CoA, H+ in cytosol and cross-references RHEA:10189.", "equation": "Ac-CoA + ACA-CoA + H2O → CoA-SH + bHMG-CoA + H+", "inputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-191293", "reactome_id": "R-ALL-191293", "label": "ACA-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57286", "formula": "C25H36N7O18P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-191374", "reactome_id": "R-ALL-191374", "label": "bHMG-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43074", "formula": "C27H39N7O20P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "hydroxymethylglutaryl-CoA synthase activity of HMGCS1 [cytosol]", "activity_id": "191334", "ec_numbers": [ "2.3.3.10" ], "accessions": null } ], "citations": [ { "id": "PMID:7913309", "title": "Human cytoplasmic 3-hydroxy-3-methylglutaryl coenzyme A synthase: expression, purification, and characterization of recombinant wild-type and Cys129 mutant enzymes", "journal": "Arch Biochem Biophys", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7913309/" } ], "x": 2660, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-191352", "label": "HMGCR dimer reduces bHMG-CoA to MVA", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:15991" ], "source_url": "https://reactome.org/content/detail/R-HSA-191352", "summary": "HMGCR dimer reduces bHMG-CoA to MVA is a Reactome release 97 curated reaction event. The source record connects bHMG-CoA, NADPH, H+ to MVA, CoA-SH, NADP+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:15991.", "equation": "bHMG-CoA + 2 NADPH + 2 H+ → MVA + CoA-SH + 2 NADP+", "inputs": [ { "node_id": "R-ALL-191374", "reactome_id": "R-ALL-191374", "label": "bHMG-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43074", "formula": "C27H39N7O20P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-191424", "reactome_id": "R-ALL-191424", "label": "MVA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:36464", "formula": "C6H11O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "hydroxymethylglutaryl-CoA reductase (NADPH) activity of HMGCR dimer [endoplasmic reticulum membrane]", "activity_id": "191301", "ec_numbers": [ "1.1.1.34" ], "accessions": null } ], "citations": [ { "id": "PMID:1967820", "title": "Regulation of the mevalonate pathway", "journal": "Nature", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/1967820/" }, { "id": "PMID:10698924", "title": "Crystal structure of the catalytic portion of human HMG-CoA reductase: insights into regulation of activity and catalysis", "journal": "EMBO J", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10698924/" } ], "x": 2938.666666666667, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-191380", "label": "Mevalonate is phosphorylated to mevalonate-5-phosphate", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17066" ], "source_url": "https://reactome.org/content/detail/R-HSA-191380", "summary": "Mevalonate is phosphorylated to mevalonate-5-phosphate is a Reactome release 97 curated reaction event. The source record connects MVA, ATP to MVA5P, ADP, H+ in cytosol and cross-references RHEA:17066.", "equation": "MVA + ATP → MVA5P + ADP + H+", "inputs": [ { "node_id": "R-ALL-191424", "reactome_id": "R-ALL-191424", "label": "MVA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36464", "formula": "C6H11O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-191296", "reactome_id": "R-ALL-191296", "label": "MVA5P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58146", "formula": "C6H10O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "mevalonate kinase activity of MVK dimer [cytosol]", "activity_id": "191389", "ec_numbers": [ "2.7.1.36" ], "accessions": null } ], "citations": [ { "id": "PMID:14730012", "title": "Mevalonate kinase is a cytosolic enzyme in humans", "journal": "J Cell Sci", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/14730012/" }, { "id": "PMID:11111075", "title": "Biochemical and genetic aspects of mevalonate kinase and its deficiency", "journal": "Biochim Biophys Acta", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/11111075/" }, { "id": "PMID:1377680", "title": "Molecular cloning of human mevalonate kinase and identification of a missense mutation in the genetic disease mevalonic aciduria", "journal": "J Biol Chem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1377680/" } ], "x": 2660, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-191402", "label": "Reduction of presqualene diphosphate to form squalene", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:22233" ], "source_url": "https://reactome.org/content/detail/R-HSA-191402", "summary": "Reduction of presqualene diphosphate to form squalene is a Reactome release 97 curated reaction event. The source record connects PSQPP, H+, NADPH to SQNE, NADP+, PPi in cytosol · endoplasmic reticulum membrane and cross-references RHEA:22233.", "equation": "PSQPP + H+ + NADPH → SQNE + NADP+ + PPi", "inputs": [ { "node_id": "R-ALL-191265", "reactome_id": "R-ALL-191265", "label": "PSQPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57310", "formula": "C30H49O7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-191269", "reactome_id": "R-ALL-191269", "label": "SQNE", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15440", "formula": "C30H50", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "squalene synthase [NAD(P)H] activity of FDFT:Mg2+ [endoplasmic reticulum membrane]", "activity_id": "191305", "ec_numbers": [ "2.5.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:10896663", "title": "Crystal structure of human squalene synthase. A key enzyme in cholesterol biosynthesis.", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10896663/" } ], "x": 2381.3333333333335, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-191414", "label": "MVD decarboxylates MVA5PP to IPPP", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23733" ], "source_url": "https://reactome.org/content/detail/R-HSA-191414", "summary": "MVD decarboxylates MVA5PP to IPPP is a Reactome release 97 curated reaction event. The source record connects MVA5PP, ATP to IPPP, ADP, Pi in cytosol and cross-references RHEA:23733.", "equation": "MVA5PP + ATP → IPPP + ADP + Pi", "inputs": [ { "node_id": "R-ALL-191403", "reactome_id": "R-ALL-191403", "label": "MVA5PP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57557", "formula": "C6H10O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-191362", "reactome_id": "R-ALL-191362", "label": "IPPP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:128769", "formula": "C5H9O7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "diphosphomevalonate decarboxylase activity of MVD dimer [cytosol]", "activity_id": "191297", "ec_numbers": [ "4.1.1.33" ], "accessions": null } ], "citations": [ { "id": "PMID:8626466", "title": "Molecular cloning and expression of the cDNAs encoding human and yeast mevalonate pyrophosphate decarboxylase", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8626466/" } ], "x": 2938.666666666667, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-191983", "label": "Cholesterol is hydroxylated to 25-hydroxycholesterol", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:46133" ], "source_url": "https://reactome.org/content/detail/R-HSA-191983", "summary": "Cholesterol is hydroxylated to 25-hydroxycholesterol is a Reactome release 97 curated reaction event. The source record connects CHOL, NADPH, H+ to H2O, NADP+, 25OH-CHOL in cytosol · endoplasmic reticulum membrane and cross-references RHEA:46133.", "equation": "CHOL + NADPH + H+ + O2 → H2O + NADP+ + 25OH-CHOL", "inputs": [ { "node_id": "R-ALL-176478", "reactome_id": "R-ALL-176478", "label": "CHOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16113", "formula": "C27H46O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-192148", "reactome_id": "R-ALL-192148", "label": "25OH-CHOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:42977", "formula": "C27H46O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "steroid hydroxylase activity of CH25H (Fe2+ cofactor) [endoplasmic reticulum membrane]", "activity_id": "192119", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:9852097", "title": "cDNA cloning of mouse and human cholesterol 25-hydroxylases, polytopic membrane proteins that synthesize a potent oxysterol regulator of lipid metabolism", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9852097/" } ], "x": 5008.666666666667, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-192123", "label": "CYP27A1 27-hydroxylates CHOL", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "biological-oxidations", "phase-one", "steroids" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:43837" ], "source_url": "https://reactome.org/content/detail/R-HSA-192123", "summary": "CYP27A1 27-hydroxylates CHOL is a Reactome release 97 curated reaction event. The source record connects CHOL, NADPH, H+ to 27OH-CHOL, H2O, NADP+ in mitochondrial matrix and cross-references RHEA:43837.", "equation": "CHOL + NADPH + H+ + O2 → 27OH-CHOL + H2O + NADP+", "inputs": [ { "node_id": "R-ALL-193840", "reactome_id": "R-ALL-193840", "label": "CHOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16113", "formula": "C27H46O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113533", "reactome_id": "R-ALL-113533", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-193846", "reactome_id": "R-ALL-193846", "label": "27OH-CHOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17703", "formula": "C27H46O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "oxidoreductase activity, acting on NAD(P)H as acceptor of CYP27A1 [mitochondrial matrix]", "activity_id": "192037", "ec_numbers": [ "1.6.1" ], "accessions": null } ], "citations": [ { "id": "PMID:1708392", "title": "Characterization of human sterol 27-hydroxylase. A mitochondrial cytochrome P-450 that catalyzes multiple oxidation reaction in bile acid biosynthesis.", "journal": "J Biol Chem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1708392/" } ], "x": 4451.333333333333, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-192312", "label": "Choloyl CoA reacts with glycine or taurine to form glycocholate or taurocholate", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:47101", "RHEA:14002" ], "source_url": "https://reactome.org/content/detail/R-HSA-192312", "summary": "Choloyl CoA reacts with glycine or taurine to form glycocholate or taurocholate is a Reactome release 97 curated reaction event. The source record connects choloyl-CoA, Gly, TAU to cholate bile salts, CoA-SH, H+ in peroxisomal matrix and cross-references RHEA:47101, RHEA:14002.", "equation": "choloyl-CoA + Gly, TAU → cholate bile salts + CoA-SH + H+", "inputs": [ { "node_id": "R-ALL-192356", "reactome_id": "R-ALL-192356", "label": "choloyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57373", "formula": "C45H70N7O20P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-193463", "reactome_id": "R-ALL-193463", "label": "Gly, TAU", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "peroxisomal matrix", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-193404", "reactome_id": "R-ALL-193404", "label": "cholate bile salts", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "peroxisomal matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-193514", "reactome_id": "R-ALL-193514", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193465", "reactome_id": "R-ALL-193465", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acyltransferase activity, transferring groups other than amino-acyl groups of BAAT [peroxisomal matrix]", "activity_id": "193371", "ec_numbers": [ "2.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:8034703", "title": "Glycine and taurine conjugation of bile acids by a single enzyme. Molecular cloning and expression of human liver bile acid CoA:amino acid N-acyltransferase.", "journal": "J Biol Chem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8034703/" } ], "x": 4451.333333333333, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-192315", "label": "Bile salts are translocated from the peroxisomal matrix to the cytosol", "schema_class": "BlackBoxEvent", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "peroxisomal membrane", "compartment_ids": [ "GO:0005778" ], "rhea_ids": [ "RHEA:71855", "RHEA:71851", "RHEA:71859", "RHEA:71703" ], "source_url": "https://reactome.org/content/detail/R-HSA-192315", "summary": "Bile salts are translocated from the peroxisomal matrix to the cytosol is a Reactome release 97 curated black-box event whose internal chemistry is not expanded by the source record. The source record connects bile salts to bile salts in peroxisomal membrane and cross-references RHEA:71855, RHEA:71851, RHEA:71859, RHEA:71703.", "equation": "bile salts → bile salts", "inputs": [ { "node_id": "R-ALL-193448", "reactome_id": "R-ALL-193448", "label": "bile salts", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "peroxisomal matrix", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-193416", "reactome_id": "R-ALL-193416", "label": "bile salts", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [], "citations": [ { "id": "PMID:12543708", "title": "The enzymes, regulation, and genetics of bile acid synthesis", "journal": "Annu Rev Biochem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12543708/" } ], "x": 4730, "y": 1776.125, "lod": "pathway", "collapsed": true, "transport": true }, { "id": "R-HSA-193060", "label": "CYP19A1 hydroxylates ANDST to E1", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-one", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:38196" ], "source_url": "https://reactome.org/content/detail/R-HSA-193060", "summary": "CYP19A1 hydroxylates ANDST to E1 is a Reactome release 97 curated reaction event. The source record connects ANDST, NADPH, H+ to H+, E1, NADP+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:38196.", "equation": "ANDST + 3 NADPH + 3 H+ + 3 O2 → 4 H+ + E1 + 3 NADP+ + 4 H2O + HCOOH", "inputs": [ { "node_id": "R-ALL-193079", "reactome_id": "R-ALL-193079", "label": "ANDST", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16422", "formula": "C19H26O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 3, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 3, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 3, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193066", "reactome_id": "R-ALL-193066", "label": "E1", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17263", "formula": "C18H22O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 3, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29460", "reactome_id": "R-ALL-29460", "label": "HCOOH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15740", "formula": "CHO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "steroid hydroxylase activity of CYP19A1 [endoplasmic reticulum membrane]", "activity_id": "193170", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:8076586", "title": "Aromatase cytochrome P450, the enzyme responsible for estrogen biosynthesis", "journal": "Endocr Rev", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8076586/" }, { "id": "PMID:2171939", "title": "Structural and functional characterization of human aromatase P-450 gene", "journal": "Eur J Biochem", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2171939/" } ], "x": 6521.333333333333, "y": 3573.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-193101", "label": "CYP11A1 cleaves 20a,22b-DHCHOL", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-one", "steroids" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:34344" ], "source_url": "https://reactome.org/content/detail/R-HSA-193101", "summary": "CYP11A1 cleaves 20a,22b-DHCHOL is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects 20a,22b-DHCHOL, O2, CYP11A1:FDXR:FDX1,FDX1L (red.) to PREG, ISCAL, H2O in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:34344.", "equation": "20a,22b-DHCHOL + O2 + 2 CYP11A1:FDXR:FDX1,FDX1L (red.) + 2 NADPH + 2 H+ → PREG + ISCAL + 2 H2O + 2 CYP11A1:FDXR:FDX1,FDX1L (ox.) + 2 NADP+", "inputs": [ { "node_id": "R-ALL-193086", "reactome_id": "R-ALL-193086", "label": "20a,22b-DHCHOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:1294", "formula": "C27H46O3", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113533", "reactome_id": "R-ALL-113533", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-5580249", "reactome_id": "R-HSA-5580249", "label": "CYP11A1:FDXR:FDX1,FDX1L (red.)", "role": "input", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-193164", "reactome_id": "R-ALL-193164", "label": "PREG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16581", "formula": "C21H32O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-193046", "reactome_id": "R-ALL-193046", "label": "ISCAL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17998", "formula": "C6H12O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-5580259", "reactome_id": "R-HSA-5580259", "label": "CYP11A1:FDXR:FDX1,FDX1L (ox.)", "role": "output", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "cholesterol monooxygenase (side-chain-cleaving) activity of CYP11A1:FDXR:FDX1,FDX1L (red.) [mitochondrial matrix]", "activity_id": "193154", "ec_numbers": [ "1.14.15.6" ], "accessions": null } ], "citations": [ { "id": "PMID:9578606", "title": "Expression of catalytically active human cytochrome p450scc in Escherichia coli and mutagenesis of isoleucine-462", "journal": "Arch Biochem Biophys", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9578606/" }, { "id": "PMID:3024157", "title": "Human cholesterol side-chain cleavage enzyme, P450scc: cDNA cloning, assignment of the gene to chromosome 15, and expression in the placenta", "journal": "Proc Natl Acad Sci U S A", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3024157/" }, { "id": "PMID:21636783", "title": "Structural basis for pregnenolone biosynthesis by the mitochondrial monooxygenase system", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21636783/" } ], "x": 6800, "y": 3573.75, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-193362", "label": "ABCB11 transports bile salts from cytosol to extracellular region", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:50056", "RHEA:50052", "RHEA:50064", "RHEA:50060" ], "source_url": "https://reactome.org/content/detail/R-HSA-193362", "summary": "ABCB11 transports bile salts from cytosol to extracellular region is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects bile salts, ATP, H2O to bile salts, ADP, Pi in plasma membrane and cross-references RHEA:50056, RHEA:50052, RHEA:50064, RHEA:50060.", "equation": "bile salts + ATP + H2O → bile salts + ADP + Pi + H+", "inputs": [ { "node_id": "R-ALL-193416", "reactome_id": "R-ALL-193416", "label": "bile salts", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-193479", "reactome_id": "R-ALL-193479", "label": "bile salts", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ABC-type bile acid transporter activity of ABCB11 [plasma membrane]", "activity_id": "193367", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:12404240", "title": "Functional expression of the canalicular bile salt export pump of human liver", "journal": "Gastroenterology", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12404240/" }, { "id": "PMID:12404239", "title": "The human bile salt export pump: characterization of substrate specificity and identification of inhibitors", "journal": "Gastroenterology", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12404239/" } ], "x": 4451.333333333333, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-193491", "label": "Chenodeoxycholoyl CoA reacts with glycine or taurine to form glycochenodeoxycholate or taurochenodeoxycholate", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:49789", "RHEA:49785" ], "source_url": "https://reactome.org/content/detail/R-HSA-193491", "summary": "Chenodeoxycholoyl CoA reacts with glycine or taurine to form glycochenodeoxycholate or taurochenodeoxycholate is a Reactome release 97 curated reaction event. The source record connects chenodeoxycholoyl-CoA, Gly, TAU to CoA-SH, chenodeoxycholate bile salts, H+ in peroxisomal matrix and cross-references RHEA:49789, RHEA:49785.", "equation": "chenodeoxycholoyl-CoA + Gly, TAU → CoA-SH + chenodeoxycholate bile salts + H+", "inputs": [ { "node_id": "R-ALL-193472", "reactome_id": "R-ALL-193472", "label": "chenodeoxycholoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62989", "formula": "C45H70N7O19P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-193463", "reactome_id": "R-ALL-193463", "label": "Gly, TAU", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "peroxisomal matrix", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-193514", "reactome_id": "R-ALL-193514", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193453", "reactome_id": "R-ALL-193453", "label": "chenodeoxycholate bile salts", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "peroxisomal matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-193465", "reactome_id": "R-ALL-193465", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acyltransferase activity, transferring groups other than amino-acyl groups of BAAT [peroxisomal matrix]", "activity_id": "193371", "ec_numbers": [ "2.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:8034703", "title": "Glycine and taurine conjugation of bile acids by a single enzyme. Molecular cloning and expression of human liver bile acid CoA:amino acid N-acyltransferase.", "journal": "J Biol Chem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8034703/" } ], "x": 4730, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-194079", "label": "SLCO1B3 transports ALB:(GCCA, TCCA) from extracellular region to cytosol", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:71851", "RHEA:71703", "RHEA:71704", "RHEA:71852" ], "source_url": "https://reactome.org/content/detail/R-HSA-194079", "summary": "SLCO1B3 transports ALB:(GCCA, TCCA) from extracellular region to cytosol is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects GCCA, TCCA to GCCA, TCCA in plasma membrane and cross-references RHEA:71851, RHEA:71703, RHEA:71704, RHEA:71852.", "equation": "GCCA, TCCA → GCCA, TCCA", "inputs": [ { "node_id": "R-ALL-194117", "reactome_id": "R-ALL-194117", "label": "GCCA, TCCA", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-194097", "reactome_id": "R-ALL-194097", "label": "GCCA, TCCA", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "bile acid transmembrane transporter activity of SLCO1B3 [plasma membrane]", "activity_id": "194088", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:11159893", "title": "Organic anion-transporting polypeptide B (OATP-B) and its functional comparison with three other OATPs of human liver", "journal": "Gastroenterology", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11159893/" } ], "x": 5008.666666666667, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-194083", "label": "SLCO1B1 transports ALB:(GCCA,TCCA) from extracellular region to cytosol", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:71851", "RHEA:71703", "RHEA:71704", "RHEA:71852" ], "source_url": "https://reactome.org/content/detail/R-HSA-194083", "summary": "SLCO1B1 transports ALB:(GCCA,TCCA) from extracellular region to cytosol is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects GCCA, TCCA to GCCA, TCCA in plasma membrane and cross-references RHEA:71851, RHEA:71703, RHEA:71704, RHEA:71852.", "equation": "GCCA, TCCA → GCCA, TCCA", "inputs": [ { "node_id": "R-ALL-194117", "reactome_id": "R-ALL-194117", "label": "GCCA, TCCA", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-194097", "reactome_id": "R-ALL-194097", "label": "GCCA, TCCA", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "bile acid transmembrane transporter activity of SLCO1B1 [plasma membrane]", "activity_id": "194120", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10358072", "title": "Identification of a novel gene family encoding human liver-specific organic anion transporter LST-1", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10358072/" }, { "id": "PMID:10601278", "title": "A novel human hepatic organic anion transporting polypeptide (OATP2). Identification of a liver-specific human organic anion transporting polypeptide and identification of rat and human hydroxymethylglutaryl-CoA reductase inhibitor transporters", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10601278/" }, { "id": "PMID:11159893", "title": "Organic anion-transporting polypeptide B (OATP-B) and its functional comparison with three other OATPs of human liver", "journal": "Gastroenterology", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11159893/" } ], "x": 4451.333333333333, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-194130", "label": "Transport (influx) of bile salts and acids by OATP-A", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:75051", "RHEA:71703", "RHEA:72683", "RHEA:71851", "RHEA:71855", "RHEA:71859", "RHEA:71704", "RHEA:71852", "RHEA:71856", "RHEA:71860", "RHEA:72684", "RHEA:75052" ], "source_url": "https://reactome.org/content/detail/R-HSA-194130", "summary": "Transport (influx) of bile salts and acids by OATP-A is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects bile salts and acids to bile salts and acids in plasma membrane and cross-references RHEA:75051, RHEA:71703, RHEA:72683, RHEA:71851, RHEA:71855, RHEA:71859, RHEA:71704, RHEA:71852, RHEA:71856, RHEA:71860, RHEA:72684, RHEA:75052.", "equation": "bile salts and acids → bile salts and acids", "inputs": [ { "node_id": "R-ALL-192483", "reactome_id": "R-ALL-192483", "label": "bile salts and acids", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-194156", "reactome_id": "R-ALL-194156", "label": "bile salts and acids", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "bile acid transmembrane transporter activity of SLCO1A2 [plasma membrane]", "activity_id": "194128", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:7557095", "title": "Molecular and functional characterization of an organic anion transporting polypeptide cloned from human liver", "journal": "Gastroenterology", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7557095/" } ], "x": 5391.333333333333, "y": 1727.5, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-194153", "label": "ABCC3 transports bile salts from cytosol to extracellular region", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "plasma membrane · cytosol · extracellular region", "compartment_ids": [ "GO:0005886", "GO:0005829", "GO:0005576" ], "rhea_ids": [ "RHEA:50056", "RHEA:50052", "RHEA:50060", "RHEA:50064", "RHEA:50048" ], "source_url": "https://reactome.org/content/detail/R-HSA-194153", "summary": "ABCC3 transports bile salts from cytosol to extracellular region is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects bile salts and acids, ATP to ADP, Pi, bile salts and acids in plasma membrane · cytosol · extracellular region and cross-references RHEA:50056, RHEA:50052, RHEA:50060, RHEA:50064, RHEA:50048.", "equation": "bile salts and acids + ATP → ADP + Pi + bile salts and acids", "inputs": [ { "node_id": "R-ALL-194156", "reactome_id": "R-ALL-194156", "label": "bile salts and acids", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-192483", "reactome_id": "R-ALL-192483", "label": "bile salts and acids", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "ABC-type bile acid transporter activity of ABCC3 [plasma membrane]", "activity_id": "194154", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:12704183", "title": "Differential modulation of the human liver conjugate transporters MRP2 and MRP3 by bile acids and organic anions", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12704183/" }, { "id": "PMID:10987286", "title": "Transport of amphipathic anions by human multidrug resistance protein 3", "journal": "Cancer Res", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10987286/" }, { "id": "PMID:12220224", "title": "Transport of bile acids in multidrug-resistance-protein 3-overexpressing cells co-transfected with the ileal Na+-dependent bile-acid transporter", "journal": "Biochem J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12220224/" } ], "x": 5008.666666666667, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-194632", "label": "Zymosterone (cholesta-8(9),24-dien-3-one) is reduced to zymosterol (cholesta-8(9),24-dien-3beta-ol)", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:33459", "RHEA:33460" ], "source_url": "https://reactome.org/content/detail/R-HSA-194632", "summary": "Zymosterone (cholesta-8(9),24-dien-3-one) is reduced to zymosterol (cholesta-8(9),24-dien-3beta-ol) is a Reactome release 97 curated reaction event. The source record connects ZYMONE, NADPH, H+ to ZYMOL, NADP+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:33459, RHEA:33460.", "equation": "ZYMONE + NADPH + H+ → ZYMOL + NADP+", "inputs": [ { "node_id": "R-ALL-194661", "reactome_id": "R-ALL-194661", "label": "ZYMONE", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:52386", "formula": "C27H42O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-194691", "reactome_id": "R-ALL-194691", "label": "ZYMOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18252", "formula": "C27H44O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "3-beta-hydroxysteroid 3-dehydrogenase (NADP+) activity of HSD17B7 [endoplasmic reticulum membrane]", "activity_id": "194731", "ec_numbers": [ "1.1.1.270" ], "accessions": null } ], "citations": [ { "id": "PMID:12829805", "title": "Closing the gap: identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis", "journal": "Mol. Endocrinol.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12829805/" } ], "x": 2381.3333333333335, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-194674", "label": "4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol is reduced to 4,4-dimethylcholesta-8(9),24-dien-3beta-ol [LBR]", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "nuclear envelope", "compartment_ids": [ "GO:0005635" ], "rhea_ids": [ "RHEA:18563" ], "source_url": "https://reactome.org/content/detail/R-HSA-194674", "summary": "4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol is reduced to 4,4-dimethylcholesta-8(9),24-dien-3beta-ol [LBR] is a Reactome release 97 curated reaction event. The source record connects 4,4DMCHtOL, NADPH, H+ to 14DMLANOL, NADP+ in nuclear envelope and cross-references RHEA:18563.", "equation": "4,4DMCHtOL + NADPH + H+ → 14DMLANOL + NADP+", "inputs": [ { "node_id": "R-ALL-194681", "reactome_id": "R-ALL-194681", "label": "4,4DMCHtOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17813", "formula": "C29H46O", "compartment": "nuclear envelope", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-194725", "reactome_id": "R-ALL-194725", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16474", "formula": "C21H30N7O17P3", "compartment": "nuclear envelope", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-194688", "reactome_id": "R-ALL-194688", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "nuclear envelope", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-194704", "reactome_id": "R-ALL-194704", "label": "14DMLANOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18364", "formula": "C29H48O", "compartment": "nuclear envelope", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-194668", "reactome_id": "R-ALL-194668", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18009", "formula": "C21H29N7O17P3", "compartment": "nuclear envelope", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "Delta14-sterol reductase activity of LBR [nuclear envelope]", "activity_id": "194706", "ec_numbers": [ "1.3.1.70" ], "accessions": null } ], "citations": [ { "id": "PMID:12618959", "title": "Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in the lamin B receptor gene", "journal": "Am J Hum Genet", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12618959/" } ], "x": 2660, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-194698", "label": "4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol is reduced to 4,4-dimethylcholesta-8(9),24-dien-3beta-ol [TM7SF2]", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:18561", "RHEA:18563" ], "source_url": "https://reactome.org/content/detail/R-HSA-194698", "summary": "4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol is reduced to 4,4-dimethylcholesta-8(9),24-dien-3beta-ol [TM7SF2] is a Reactome release 97 curated reaction event. The source record connects 4,4DMCHtOL, NADPH, H+ to 14DMLANOL, NADP+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:18561, RHEA:18563.", "equation": "4,4DMCHtOL + NADPH + H+ → 14DMLANOL + NADP+", "inputs": [ { "node_id": "R-ALL-194644", "reactome_id": "R-ALL-194644", "label": "4,4DMCHtOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17813", "formula": "C29H46O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-194633", "reactome_id": "R-ALL-194633", "label": "14DMLANOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18364", "formula": "C29H48O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "Delta14-sterol reductase activity of TM7SF2 [endoplasmic reticulum membrane]", "activity_id": "194714", "ec_numbers": [ "1.3.1.70" ], "accessions": null } ], "citations": [ { "id": "PMID:16784888", "title": "Sterol dependent regulation of human TM7SF2 gene expression: role of the encoded 3beta-hydroxysterol Delta14-reductase in human cholesterol biosynthesis", "journal": "Biochim Biophys Acta", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16784888/" } ], "x": 2938.666666666667, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-195664", "label": "Cholesta-7,24-dien-3beta-ol is desaturated to form cholesta-5,7,24-trien-3beta-ol", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:34003", "RHEA:34004" ], "source_url": "https://reactome.org/content/detail/R-HSA-195664", "summary": "Cholesta-7,24-dien-3beta-ol is desaturated to form cholesta-5,7,24-trien-3beta-ol is a Reactome release 97 curated reaction event. The source record connects CHdOL, NADPH, H+ to 7dhDESOL, NADP+, H2O in cytosol · endoplasmic reticulum membrane and cross-references RHEA:34003, RHEA:34004.", "equation": "CHdOL + NADPH + H+ + O2 → 7dhDESOL + NADP+ + 2 H2O", "inputs": [ { "node_id": "R-ALL-195691", "reactome_id": "R-ALL-195691", "label": "CHdOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16290", "formula": "C27H44O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-195655", "reactome_id": "R-ALL-195655", "label": "7dhDESOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:27910", "formula": "C27H42O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "C-5 sterol desaturase activity of SC5D [endoplasmic reticulum membrane]", "activity_id": "195666", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:12189593", "title": "Lathosterolosis, a novel multiple-malformation/mental retardation syndrome due to deficiency of 3beta-hydroxysteroid-delta5-desaturase", "journal": "Am J Hum Genet", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12189593/" }, { "id": "PMID:12812989", "title": "Lathosterolosis: an inborn error of human and murine cholesterol synthesis due to lathosterol 5-desaturase deficiency", "journal": "Hum Mol Genet", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12812989/" } ], "x": 2381.3333333333335, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-196402", "label": "Cholesta-5,7,24-trien-3beta-ol is reduced to desmosterol", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:46740", "RHEA:46741" ], "source_url": "https://reactome.org/content/detail/R-HSA-196402", "summary": "Cholesta-5,7,24-trien-3beta-ol is reduced to desmosterol is a Reactome release 97 curated reaction event. The source record connects 7dhDESOL, NADPH, H+ to DESMOL, NADP+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:46740, RHEA:46741.", "equation": "7dhDESOL + NADPH + H+ → DESMOL + NADP+", "inputs": [ { "node_id": "R-ALL-195655", "reactome_id": "R-ALL-195655", "label": "7dhDESOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:27910", "formula": "C27H42O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-196420", "reactome_id": "R-ALL-196420", "label": "DESMOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17737", "formula": "C27H44O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "7-dehydrocholesterol reductase activity of DHCR7 [endoplasmic reticulum membrane]", "activity_id": "196403", "ec_numbers": [ "1.3.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:9465114", "title": "Molecular cloning and expression of the human delta7-sterol reductase", "journal": "Proc Natl Acad Sci U S A", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9465114/" } ], "x": 2381.3333333333335, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-196417", "label": "Reduction of desmosterol to cholesterol", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:36391", "RHEA:36393" ], "source_url": "https://reactome.org/content/detail/R-HSA-196417", "summary": "Reduction of desmosterol to cholesterol is a Reactome release 97 curated reaction event. The source record connects DESMOL, NADPH, H+ to CHOL, NADP+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:36391, RHEA:36393.", "equation": "DESMOL + NADPH + H+ → CHOL + NADP+", "inputs": [ { "node_id": "R-ALL-196420", "reactome_id": "R-ALL-196420", "label": "DESMOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17737", "formula": "C27H44O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-193384", "reactome_id": "R-ALL-193384", "label": "CHOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16113", "formula": "C27H46O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "Delta24-sterol reductase activity of DHCR24 [endoplasmic reticulum membrane]", "activity_id": "196404", "ec_numbers": [ "1.3.1.72" ], "accessions": null } ], "citations": [ { "id": "PMID:11519011", "title": "Mutations in the 3beta-hydroxysterol Delta24-reductase gene cause desmosterolosis, an autosomal recessive disorder of cholesterol biosynthesis", "journal": "Am J Hum Genet", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11519011/" } ], "x": 2660, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-197186", "label": "NAPRT1 dimer transfers PRIB to NCA to form NAMN", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:36164" ], "source_url": "https://reactome.org/content/detail/R-HSA-197186", "summary": "NAPRT1 dimer transfers PRIB to NCA to form NAMN is a Reactome release 97 curated reaction event. The source record connects NCA, PRPP, ATP to PPi, NAMN, ADP in cytosol and cross-references RHEA:36164.", "equation": "NCA + PRPP + ATP + H2O → PPi + NAMN + ADP + Pi", "inputs": [ { "node_id": "R-ALL-197230", "reactome_id": "R-ALL-197230", "label": "NCA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:32544", "formula": "C6H4NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73566", "reactome_id": "R-ALL-73566", "label": "PRPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58017", "formula": "C5H8O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8869359", "reactome_id": "R-ALL-8869359", "label": "NAMN", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57502", "formula": "C11H12NO9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "nicotinate phosphoribosyltransferase activity of NAPRT1 dimer [cytosol]", "activity_id": "197236", "ec_numbers": [ "6.3.4.21" ], "accessions": null } ], "citations": [ { "id": "PMID:4349867", "title": "Nicotinate phosphoribosyltransferase of human erythrocytes. Purification and properties.", "journal": "J Biol Chem", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4349867/" }, { "id": "PMID:13563527", "title": "Biosynthesis of diphosphopyridine nucleotide. II. Enzymatic aspects.", "journal": "J Biol Chem", "year": 1958, "url": "https://pubmed.ncbi.nlm.nih.gov/13563527/" }, { "id": "PMID:17604275", "title": "Elevation of cellular NAD levels by nicotinic acid and involvement of nicotinic acid phosphoribosyltransferase in human cells", "journal": "J Biol Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17604275/" } ], "x": 5948.666666666667, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-197198", "label": "NADK:Zn2+ tetramer phosphorylates NAD+ to NADP+", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18630" ], "source_url": "https://reactome.org/content/detail/R-HSA-197198", "summary": "NADK:Zn2+ tetramer phosphorylates NAD+ to NADP+ is a Reactome release 97 curated reaction event. The source record connects NAD+, ATP to NADP+, ADP, H+ in cytosol and cross-references RHEA:18630.", "equation": "NAD+ + ATP → NADP+ + ADP + H+", "inputs": [ { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "NAD+ kinase activity of NADK:Zn2+ tetramer [cytosol]", "activity_id": "197237", "ec_numbers": [ "2.7.1.23" ], "accessions": null } ], "citations": [ { "id": "PMID:11594753", "title": "Structural and functional characterization of human NAD kinase", "journal": "Biochem Biophys Res Commun", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11594753/" } ], "x": 5670, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-197235", "label": "NMNAT2 transfers an adenylyl group from ATP to NAMN to yield NAAD", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "cytosol · Golgi membrane", "compartment_ids": [ "GO:0005829", "GO:0000139" ], "rhea_ids": [ "RHEA:22861" ], "source_url": "https://reactome.org/content/detail/R-HSA-197235", "summary": "NMNAT2 transfers an adenylyl group from ATP to NAMN to yield NAAD is a Reactome release 97 curated reaction event. The source record connects ATP, NAMN, H+ to NAAD, PPi in cytosol · Golgi membrane and cross-references RHEA:22861.", "equation": "ATP + NAMN + H+ → NAAD + PPi", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-8869359", "reactome_id": "R-ALL-8869359", "label": "NAMN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57502", "formula": "C11H12NO9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-197256", "reactome_id": "R-ALL-197256", "label": "NAAD", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58437", "formula": "C21H24N6O15P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "nicotinate-nucleotide adenylyltransferase activity of NMNAT2:Mg2+ [Golgi membrane]", "activity_id": "197217", "ec_numbers": [ "2.7.7.18" ], "accessions": null } ], "citations": [ { "id": "PMID:14516279", "title": "Characterization of human brain nicotinamide 5'-mononucleotide adenylyltransferase-2 and expression in human pancreas", "journal": "Biochem J", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/14516279/" }, { "id": "PMID:12359228", "title": "Identification of a novel human nicotinamide mononucleotide adenylyltransferase", "journal": "Biochem Biophys Res Commun", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12359228/" }, { "id": "PMID:16118205", "title": "Subcellular compartmentation and differential catalytic properties of the three human nicotinamide mononucleotide adenylyltransferase isoforms", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16118205/" }, { "id": "PMID:17402747", "title": "Initial-rate kinetics of human NMN-adenylyltransferases: substrate and metal ion specificity, inhibition by products and multisubstrate analogues, and isozyme contributions to NAD+ biosynthesis", "journal": "Biochemistry", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17402747/" } ], "x": 5948.666666666667, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-197268", "label": "QPRT transfers PRIB to QUIN to form NAMN", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:12735" ], "source_url": "https://reactome.org/content/detail/R-HSA-197268", "summary": "QPRT transfers PRIB to QUIN to form NAMN is a Reactome release 97 curated reaction event. The source record connects QUIN, PRPP, H+ to PPi, CO2, NAMN in cytosol and cross-references RHEA:12735.", "equation": "QUIN + PRPP + 2 H+ → PPi + CO2 + NAMN", "inputs": [ { "node_id": "R-ALL-197238", "reactome_id": "R-ALL-197238", "label": "QUIN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29959", "formula": "C7H3NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73566", "reactome_id": "R-ALL-73566", "label": "PRPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58017", "formula": "C5H8O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8869359", "reactome_id": "R-ALL-8869359", "label": "NAMN", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57502", "formula": "C11H12NO9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "nicotinate-nucleotide diphosphorylase (carboxylating) activity of QPRT [cytosol]", "activity_id": "197214", "ec_numbers": [ "2.4.2.19" ], "accessions": null } ], "citations": [ { "id": "PMID:9473669", "title": "Characterization and functional expression of the cDNA encoding human brain quinolinate phosphoribosyltransferase", "journal": "Biochim Biophys Acta", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9473669/" }, { "id": "PMID:17868694", "title": "Structural and kinetic characterization of quinolinate phosphoribosyltransferase (hQPRTase) from homo sapiens", "journal": "J Mol Biol", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17868694/" } ], "x": 5391.333333333333, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-197271", "label": "NADSYN1 hexamer amidates NAAD to NAD+", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24385" ], "source_url": "https://reactome.org/content/detail/R-HSA-197271", "summary": "NADSYN1 hexamer amidates NAAD to NAD+ is a Reactome release 97 curated reaction event. The source record connects NAAD, ATP, H2O to NAD+, AMP, PPi in cytosol and cross-references RHEA:24385.", "equation": "NAAD + ATP + H2O + L-Gln → NAD+ + AMP + PPi + L-Glu + H+", "inputs": [ { "node_id": "R-ALL-197256", "reactome_id": "R-ALL-197256", "label": "NAAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58437", "formula": "C21H24N6O15P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29472", "reactome_id": "R-ALL-29472", "label": "L-Gln", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58359", "formula": "C5H10N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "NAD+ synthase (glutamine-hydrolyzing) activity of NADSYN1 hexamer [cytosol]", "activity_id": "197281", "ec_numbers": [ "6.3.5.1" ], "accessions": null } ], "citations": [ { "id": "PMID:12547821", "title": "Molecular identification of human glutamine- and ammonia-dependent NAD synthetases. Carbon-nitrogen hydrolase domain confers glutamine dependency.", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12547821/" } ], "x": 5670, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-197963", "label": "DHFR2 reduces FOLA to DHF", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:31105" ], "source_url": "https://reactome.org/content/detail/R-HSA-197963", "summary": "DHFR2 reduces FOLA to DHF is a Reactome release 97 curated reaction event. The source record connects FOLA, NADPH, H+ to DHF, NADP+ in cytosol and cross-references RHEA:31105.", "equation": "FOLA + NADPH + H+ → DHF + NADP+", "inputs": [ { "node_id": "R-ALL-197965", "reactome_id": "R-ALL-197965", "label": "FOLA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62501", "formula": "C19H17N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-73604", "reactome_id": "R-ALL-73604", "label": "DHF", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57451", "formula": "C19H19N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "folate reductase activity of DHFR2 [cytosol]", "activity_id": "197966", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:6323448", "title": "The functional human dihydrofolate reductase gene", "journal": "J Biol Chem", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6323448/" } ], "x": 4451.333333333333, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-197972", "label": "DHF is reduced to tetrahydrofolate (THF)", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15011" ], "source_url": "https://reactome.org/content/detail/R-HSA-197972", "summary": "DHF is reduced to tetrahydrofolate (THF) is a Reactome release 97 curated reaction event. The source record connects DHF, NADPH, H+ to THF, NADP+ in cytosol and cross-references RHEA:15011.", "equation": "DHF + NADPH + H+ → THF + NADP+", "inputs": [ { "node_id": "R-ALL-73604", "reactome_id": "R-ALL-73604", "label": "DHF", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57451", "formula": "C19H19N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111355", "reactome_id": "R-ALL-111355", "label": "THF", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57453", "formula": "C19H21N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "folate reductase activity of DHFR2 [cytosol]", "activity_id": "197966", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:6323448", "title": "The functional human dihydrofolate reductase gene", "journal": "J Biol Chem", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6323448/" }, { "id": "PMID:2248959", "title": "Crystal structures of recombinant human dihydrofolate reductase complexed with folate and 5-deazafolate", "journal": "Biochemistry", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2248959/" } ], "x": 4730, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-198513", "label": "SLC7A4 exchanges G6P for Pi across the ER membrane", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol · endoplasmic reticulum membrane · endoplasmic reticulum lumen", "compartment_ids": [ "GO:0005829", "GO:0005789", "GO:0005788" ], "rhea_ids": [ "RHEA:71537" ], "source_url": "https://reactome.org/content/detail/R-HSA-198513", "summary": "SLC7A4 exchanges G6P for Pi across the ER membrane is a Reactome release 97 curated reaction event. The source record connects G6P, Pi to G6P, Pi in cytosol · endoplasmic reticulum membrane · endoplasmic reticulum lumen and cross-references RHEA:71537.", "equation": "G6P + Pi → G6P + Pi", "inputs": [ { "node_id": "R-ALL-30537", "reactome_id": "R-ALL-30537", "label": "G6P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58225", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113551", "reactome_id": "R-ALL-113551", "label": "Pi", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113782", "reactome_id": "R-ALL-113782", "label": "G6P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58225", "formula": "C6H11O9P", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glucose 6-phosphate:phosphate antiporter activity of SLC37A4 [endoplasmic reticulum membrane]", "activity_id": "198473", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:18337460", "title": "The glucose-6-phosphate transporter is a phosphate-linked antiporter deficient in glycogen storage disease type Ib and Ic", "journal": "FASEB J.", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18337460/" }, { "id": "PMID:9428641", "title": "Sequence of a putative glucose 6-phosphate translocase, mutated in glycogen storage disease type Ib", "journal": "FEBS Lett", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9428641/" }, { "id": "PMID:9758626", "title": "A gene on chromosome 11q23 coding for a putative glucose- 6-phosphate translocase is mutated in glycogen-storage disease types Ib and Ic", "journal": "Am. J. Hum. Genet.", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9758626/" } ], "x": 1808.6666666666667, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-198563", "label": "IDO1 dioxygenates L-Trp to NFK", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24537" ], "source_url": "https://reactome.org/content/detail/R-HSA-198563", "summary": "IDO1 dioxygenates L-Trp to NFK is a Reactome release 97 curated reaction event. The source record connects L-Trp, O2 to NFK in cytosol and cross-references RHEA:24537.", "equation": "L-Trp + O2 → NFK", "inputs": [ { "node_id": "R-ALL-29500", "reactome_id": "R-ALL-29500", "label": "L-Trp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57912", "formula": "C11H12N2O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-33179", "reactome_id": "R-ALL-33179", "label": "NFK", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58629", "formula": "C11H12N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-tryptophan 2,3-dioxygenase activity of IDO1 [cytosol]", "activity_id": "198570", "ec_numbers": [ "1.13.11.11" ], "accessions": null } ], "citations": [ { "id": "PMID:16477023", "title": "Crystal structure of human indoleamine 2,3-dioxygenase: catalytic mechanism of O2 incorporation by a heme-containing dioxygenase", "journal": "Proc Natl Acad Sci USA", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16477023/" } ], "x": 8018.666666666667, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-199803", "label": "RPE dimers isomerize XY5P to RU5P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13679" ], "source_url": "https://reactome.org/content/detail/R-HSA-199803", "summary": "RPE dimers isomerize XY5P to RU5P is a Reactome release 97 curated reaction event. The source record connects XY5P to RU5P in cytosol and cross-references RHEA:13679.", "equation": "XY5P → RU5P", "inputs": [ { "node_id": "R-ALL-29790", "reactome_id": "R-ALL-29790", "label": "XY5P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57737", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29732", "reactome_id": "R-ALL-29732", "label": "RU5P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58121", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "D-ribulose-phosphate 3-epimerase activity of RPE:Fe2+ dimers [cytosol]", "activity_id": "71302", "ec_numbers": [ "5.1.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:2581946", "title": "Phosphoribosylpyrophosphate synthesis from glucose decreases during amino acid starvation of human lymphoblasts", "journal": "J Biol Chem", "year": 1985, "url": "https://pubmed.ncbi.nlm.nih.gov/2581946/" }, { "id": "PMID:7396409", "title": "Biochemical genetics of the pentose phosphate cycle: human ribose 5-phosphate isomerase (RPI) and ribulose 5-phosphate 3-epimerase (RPE)", "journal": "Ann Hum Genet", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/7396409/" } ], "x": 590, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200406", "label": "CPT1A transfers PALM to CAR", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "cytosol · mitochondrial outer membrane · mitochondrial intermembrane space", "compartment_ids": [ "GO:0005829", "GO:0005741", "GO:0005758" ], "rhea_ids": [ "RHEA:12661", "RHEA:12662" ], "source_url": "https://reactome.org/content/detail/R-HSA-200406", "summary": "CPT1A transfers PALM to CAR is a Reactome release 97 curated reaction event. The source record connects CAR, PALM-CoA to CoA-SH, L-PCAR in cytosol · mitochondrial outer membrane · mitochondrial intermembrane space and cross-references RHEA:12661, RHEA:12662.", "equation": "CAR + PALM-CoA → CoA-SH + L-PCAR", "inputs": [ { "node_id": "R-ALL-549207", "reactome_id": "R-ALL-549207", "label": "CAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-200993", "reactome_id": "R-ALL-200993", "label": "PALM-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57379", "formula": "C37H62N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-201034", "reactome_id": "R-ALL-201034", "label": "L-PCAR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17490", "formula": "C23H45NO4", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carnitine O-palmitoyltransferase activity of CPT1A [mitochondrial outer membrane]", "activity_id": "201041", "ec_numbers": [ "2.3.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:14517221", "title": "Functional and structural basis of carnitine palmitoyltransferase 1A deficiency", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14517221/" }, { "id": "PMID:9691089", "title": "Molecular basis of hepatic carnitine palmitoyltransferase I deficiency", "journal": "J Clin Invest", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9691089/" }, { "id": "PMID:11790793", "title": "Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: mutational analysis of a malonyl-CoA affinity domain", "journal": "J Biol Chem", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11790793/" }, { "id": "PMID:14711372", "title": "Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal", "journal": "Biochem J", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/14711372/" }, { "id": "PMID:11356169", "title": "Structure-function relationships of the liver and muscle isoforms of carnitine palmitoyltransferase I", "journal": "Biochem Soc Trans", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11356169/" }, { "id": "PMID:9344464", "title": "Functional studies of yeast-expressed human heart muscle carnitine palmitoyltransferase I", "journal": "Arch Biochem Biophys", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9344464/" } ], "x": 590, "y": 2076.8333333333335, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200410", "label": "CPT2 converts acylcarnitine to acyl-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:12663", "RHEA:40281" ], "source_url": "https://reactome.org/content/detail/R-HSA-200410", "summary": "CPT2 converts acylcarnitine to acyl-CoA is a Reactome release 97 curated reaction event. The source record connects CoA-SH, C8-C18 Acyl-CAR to CAR, C8-C18 Acyl-CoA in mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:12663, RHEA:40281.", "equation": "CoA-SH + C8-C18 Acyl-CAR → CAR + C8-C18 Acyl-CoA", "inputs": [ { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9971105", "reactome_id": "R-ALL-9971105", "label": "C8-C18 Acyl-CAR", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-201023", "reactome_id": "R-ALL-201023", "label": "CAR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9971103", "reactome_id": "R-ALL-9971103", "label": "C8-C18 Acyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "carnitine O-acyltransferase activity of CPT2 [mitochondrial inner membrane]", "activity_id": "201039", "ec_numbers": [ "2.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:7711730", "title": "Carnitine palmitoyltransferase II deficiency: structure of the gene and characterization of two novel disease-causing mutations", "journal": "Hum Mol Genet", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7711730/" }, { "id": "PMID:20538056", "title": "Carnitine palmitoyltransferase 2: New insights on the substrate specificity and implications for acylcarnitine profiling", "journal": "Biochim Biophys Acta", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20538056/" } ], "x": 590, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200423", "label": "pAMPK inactivates ACACB, inhibiting malonyl-CoA synthesis", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "cytosol · mitochondrial outer membrane", "compartment_ids": [ "GO:0005829", "GO:0005741" ], "rhea_ids": [ "RHEA:17990" ], "source_url": "https://reactome.org/content/detail/R-HSA-200423", "summary": "pAMPK inactivates ACACB, inhibiting malonyl-CoA synthesis is a Reactome release 97 curated reaction event. The source record connects Btn-ACACB:2Mn2+ polymer, ATP to Btn-p-S222-ACACB:2Mn2+ polymer, ADP, H+ in cytosol · mitochondrial outer membrane and cross-references RHEA:17990.", "equation": "Btn-ACACB:2Mn2+ polymer + ATP → Btn-p-S222-ACACB:2Mn2+ polymer + ADP + H+", "inputs": [ { "node_id": "R-HSA-8867341", "reactome_id": "R-HSA-8867341", "label": "Btn-ACACB:2Mn2+ polymer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial outer membrane", "entity_kind": "Polymer", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-HSA-8876893", "reactome_id": "R-HSA-8876893", "label": "Btn-p-S222-ACACB:2Mn2+ polymer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial outer membrane", "entity_kind": "Polymer", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "AMP-activated protein kinase activity of AMPK heterotrimer (active) [cytosol]", "activity_id": "200412", "ec_numbers": [ "2.7.11.1" ], "accessions": null } ], "citations": [ { "id": "PMID:15060529", "title": "AMP kinase and malonyl-CoA: targets for therapy of the metabolic syndrome", "journal": "Nat Rev Drug Discov", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15060529/" } ], "x": 868.6666666666667, "y": 2206.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200424", "label": "Exchange of acylcarnitine and carnitine across the inner mitochondrial membrane", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "mitochondrial intermembrane space · mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005758", "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:49916", "RHEA:49926" ], "source_url": "https://reactome.org/content/detail/R-HSA-200424", "summary": "Exchange of acylcarnitine and carnitine across the inner mitochondrial membrane is a Reactome release 97 curated reaction event. The source record connects CAR, Acyl-CAR to CAR, Acyl-CAR in mitochondrial intermembrane space · mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:49916, RHEA:49926.", "equation": "CAR + Acyl-CAR → CAR + Acyl-CAR", "inputs": [ { "node_id": "R-ALL-201023", "reactome_id": "R-ALL-201023", "label": "CAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9971099", "reactome_id": "R-ALL-9971099", "label": "Acyl-CAR", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial intermembrane space", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-549207", "reactome_id": "R-ALL-549207", "label": "CAR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9971097", "reactome_id": "R-ALL-9971097", "label": "Acyl-CAR", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "carnitine:O-acyl-L-carnitine antiporter activity of SLC25A20 [mitochondrial inner membrane]", "activity_id": "200567", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:9399886", "title": "Cloning of the human carnitine-acylcarnitine carrier cDNA and identification of the molecular defect in a patient", "journal": "Am J Hum Genet", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9399886/" } ], "x": 311.33333333333337, "y": 2206.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200474", "label": "NMNAT3 transfers an adenylyl group from ATP to NAMN to yield NAAD", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:22861", "RHEA:21361" ], "source_url": "https://reactome.org/content/detail/R-HSA-200474", "summary": "NMNAT3 transfers an adenylyl group from ATP to NAMN to yield NAAD is a Reactome release 97 curated reaction event. The source record connects NAMN, ATP, H+ to NAAD, PPi in mitochondrial matrix and cross-references RHEA:22861, RHEA:21361.", "equation": "NAMN + ATP + H+ → NAAD + PPi", "inputs": [ { "node_id": "R-ALL-8938083", "reactome_id": "R-ALL-8938083", "label": "NAMN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57502", "formula": "C11H12NO9P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-8938090", "reactome_id": "R-ALL-8938090", "label": "NAAD", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58437", "formula": "C21H24N6O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "nicotinate-nucleotide adenylyltransferase activity of 4xNMNAT3:4xMg2+ [mitochondrial matrix]", "activity_id": "200494", "ec_numbers": [ "2.7.7.18" ], "accessions": null } ], "citations": [ { "id": "PMID:16118205", "title": "Subcellular compartmentation and differential catalytic properties of the three human nicotinamide mononucleotide adenylyltransferase isoforms", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16118205/" }, { "id": "PMID:17402747", "title": "Initial-rate kinetics of human NMN-adenylyltransferases: substrate and metal ion specificity, inhibition by products and multisubstrate analogues, and isozyme contributions to NAD+ biosynthesis", "journal": "Biochemistry", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17402747/" }, { "id": "PMID:12574164", "title": "Structural characterization of a human cytosolic NMN/NaMN adenylyltransferase and implication in human NAD biosynthesis", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12574164/" } ], "x": 5391.333333333333, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200512", "label": "NMNAT1 transfers an adenylyl group from ATP to NAMN to yield NAAD", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "nucleoplasm", "compartment_ids": [ "GO:0005654" ], "rhea_ids": [ "RHEA:22861", "RHEA:21361" ], "source_url": "https://reactome.org/content/detail/R-HSA-200512", "summary": "NMNAT1 transfers an adenylyl group from ATP to NAMN to yield NAAD is a Reactome release 97 curated reaction event. The source record connects NAMN, ATP, H+ to NAAD, PPi in nucleoplasm and cross-references RHEA:22861, RHEA:21361.", "equation": "NAMN + ATP + H+ → NAAD + PPi", "inputs": [ { "node_id": "R-ALL-200492", "reactome_id": "R-ALL-200492", "label": "NAMN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57502", "formula": "C11H12NO9P", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29358", "reactome_id": "R-ALL-29358", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9668967", "reactome_id": "R-ALL-9668967", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-200499", "reactome_id": "R-ALL-200499", "label": "NAAD", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58437", "formula": "C21H24N6O15P2", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113541", "reactome_id": "R-ALL-113541", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "nicotinate-nucleotide adenylyltransferase activity of 6xNMNAT1:6xZn2+ [nucleoplasm]", "activity_id": "200481", "ec_numbers": [ "2.7.7.18" ], "accessions": null } ], "citations": [ { "id": "PMID:16118205", "title": "Subcellular compartmentation and differential catalytic properties of the three human nicotinamide mononucleotide adenylyltransferase isoforms", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16118205/" }, { "id": "PMID:17402747", "title": "Initial-rate kinetics of human NMN-adenylyltransferases: substrate and metal ion specificity, inhibition by products and multisubstrate analogues, and isozyme contributions to NAD+ biosynthesis", "journal": "Biochemistry", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17402747/" }, { "id": "PMID:11248244", "title": "Characterization of recombinant human nicotinamide mononucleotide adenylyl transferase (NMNAT), a nuclear enzyme essential for NAD synthesis", "journal": "FEBS Lett", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11248244/" }, { "id": "PMID:11788603", "title": "Structure of human nicotinamide/nicotinic acid mononucleotide adenylyltransferase. Basis for the dual substrate specificity and activation of the oncolytic agent tiazofurin.", "journal": "J Biol Chem", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11788603/" } ], "x": 5670, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200555", "label": "Formation of Malonyl-CoA from Acetyl-CoA (liver)", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11309" ], "source_url": "https://reactome.org/content/detail/R-HSA-200555", "summary": "Formation of Malonyl-CoA from Acetyl-CoA (liver) is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, ATP, HCO3- to ADP, Pi, Mal-CoA in cytosol and cross-references RHEA:11309.", "equation": "Ac-CoA + ATP + HCO3- → ADP + Pi + Mal-CoA + H+", "inputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111627", "reactome_id": "R-ALL-111627", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29508", "reactome_id": "R-ALL-29508", "label": "Mal-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57384", "formula": "C24H33N7O19P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acetyl-CoA carboxylase activity of Btn-ACACA [cytosol]", "activity_id": "200565", "ec_numbers": [ "6.4.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:7732023", "title": "Human acetyl-CoA carboxylase: characterization, molecular cloning, and evidence for two isoforms", "journal": "Proc Natl Acad Sci U S A", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7732023/" }, { "id": "PMID:16854592", "title": "Expression, purification, and characterization of human and rat acetyl coenzyme A carboxylase (ACC) isozymes", "journal": "Protein Expr Purif", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/16854592/" }, { "id": "PMID:18455495", "title": "Differential activation of recombinant human acetyl-CoA carboxylases 1 and 2 by citrate", "journal": "Arch Biochem Biophys", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18455495/" } ], "x": 1530, "y": 1727.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-200646", "label": "Cytosolic folate export across the plasma membrane", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:70159" ], "source_url": "https://reactome.org/content/detail/R-HSA-200646", "summary": "Cytosolic folate export across the plasma membrane is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects FOLA, H+ to FOLA, H+ in plasma membrane and cross-references RHEA:70159.", "equation": "FOLA + H+ → FOLA + H+", "inputs": [ { "node_id": "R-ALL-197965", "reactome_id": "R-ALL-197965", "label": "FOLA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62501", "formula": "C19H17N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-200716", "reactome_id": "R-ALL-200716", "label": "FOLA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62501", "formula": "C19H17N7O6", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "folic acid:proton symporter activity of SLC46A1 [plasma membrane]", "activity_id": "200706", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:8662720", "title": "Purification and properties of human cytosolic folylpoly-gamma-glutamate synthetase and organization, localization, and differential splicing of its gene", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8662720/" }, { "id": "PMID:17129779", "title": "Identification of an intestinal folate transporter and the molecular basis for hereditary folate malabsorption", "journal": "Cell", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17129779/" }, { "id": "PMID:17962486", "title": "Cloning and functional characterization of the proton-coupled electrogenic folate transporter and analysis of its expression in retinal cell types", "journal": "Invest Ophthalmol Vis Sci", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17962486/" } ], "x": 4730, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-200729", "label": "SLC46A1 transports FOLA from extracellular region to cytosol", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:70159" ], "source_url": "https://reactome.org/content/detail/R-HSA-200729", "summary": "SLC46A1 transports FOLA from extracellular region to cytosol is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects FOLA, H+ to FOLA, H+ in plasma membrane and cross-references RHEA:70159.", "equation": "FOLA + H+ → FOLA + H+", "inputs": [ { "node_id": "R-ALL-200716", "reactome_id": "R-ALL-200716", "label": "FOLA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62501", "formula": "C19H17N7O6", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-197965", "reactome_id": "R-ALL-197965", "label": "FOLA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62501", "formula": "C19H17N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "folic acid:proton symporter activity of SLC46A1 [plasma membrane]", "activity_id": "200706", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:8662720", "title": "Purification and properties of human cytosolic folylpoly-gamma-glutamate synthetase and organization, localization, and differential splicing of its gene", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8662720/" }, { "id": "PMID:17129779", "title": "Identification of an intestinal folate transporter and the molecular basis for hereditary folate malabsorption", "journal": "Cell", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17129779/" }, { "id": "PMID:17962486", "title": "Cloning and functional characterization of the proton-coupled electrogenic folate transporter and analysis of its expression in retinal cell types", "journal": "Invest Ophthalmol Vis Sci", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17962486/" } ], "x": 5008.666666666667, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-201035", "label": "ACSL1,3,5,6 ligate CoA to PALM to form PALM-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:30752" ], "source_url": "https://reactome.org/content/detail/R-HSA-201035", "summary": "ACSL1,3,5,6 ligate CoA to PALM to form PALM-CoA is a Reactome release 97 curated reaction event. The source record connects ATP, CoA-SH, PALM to AMP, H2O, PALM-CoA in cytosol · endoplasmic reticulum membrane and cross-references RHEA:30752.", "equation": "ATP + CoA-SH + PALM → AMP + H2O + PALM-CoA + PPi", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-3632881", "reactome_id": "R-ALL-3632881", "label": "PALM", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:7896", "formula": "C16H31O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-200993", "reactome_id": "R-ALL-200993", "label": "PALM-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57379", "formula": "C37H62N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "long-chain fatty acid-CoA ligase activity of ACSL1,3,5,6 [endoplasmic reticulum membrane]", "activity_id": "200992", "ec_numbers": [ "6.2.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:10548543", "title": "Identification and molecular characterization of acyl-CoA synthetase in human erythrocytes and erythroid precursors", "journal": "Biochem J", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10548543/" }, { "id": "PMID:17379924", "title": "Involvement of ACSL in local synthesis of neutral lipids in cytoplasmic lipid droplets in human hepatocyte HuH7", "journal": "J Lipid Res", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17379924/" }, { "id": "PMID:17681178", "title": "Regulation of enterocyte apoptosis by acyl-CoA synthetase 5 splicing", "journal": "Gastroenterology", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17681178/" } ], "x": 1808.6666666666667, "y": 1727.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2090085", "label": "HGSNAT oligomer acetylates Heparan chain(1)", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "lysosomal membrane · cytosol · lysosomal lumen", "compartment_ids": [ "GO:0005765", "GO:0005829", "GO:0043202" ], "rhea_ids": [ "RHEA:15126" ], "source_url": "https://reactome.org/content/detail/R-HSA-2090085", "summary": "HGSNAT oligomer acetylates Heparan chain(1) is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, GlcN- group to CoA-SH, GlcNAc- group, H+ in lysosomal membrane · cytosol · lysosomal lumen and cross-references RHEA:15126.", "equation": "Ac-CoA + GlcN- group → CoA-SH + GlcNAc- group + H+", "inputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2090045", "reactome_id": "R-ALL-2090045", "label": "GlcN- group", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:63811", "formula": "C29H48NO24", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-2090053", "reactome_id": "R-ALL-2090053", "label": "GlcNAc- group", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:747401", "formula": "C31H50NO26", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-428040", "reactome_id": "R-ALL-428040", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "heparan-alpha-glucosaminide N-acetyltransferase activity of HGSNAT oligomer [lysosomal membrane]", "activity_id": "1678732", "ec_numbers": [ "2.3.1.78" ], "accessions": null } ], "citations": [ { "id": "PMID:20650889", "title": "Analysis of the biogenesis of heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase provides insights into the mechanism underlying its complete deficiency in mucopolysaccharidosis IIIC", "journal": "J Biol Chem", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20650889/" }, { "id": "PMID:16960811", "title": "Identification of the gene encoding the enzyme deficient in mucopolysaccharidosis IIIC (Sanfilippo disease type C)", "journal": "Am J Hum Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16960811/" }, { "id": "PMID:17033958", "title": "Mutations in TMEM76* cause mucopolysaccharidosis IIIC (Sanfilippo C syndrome)", "journal": "Am J Hum Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17033958/" } ], "x": 2381.3333333333335, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2160492", "label": "IL4I1:FAD oxidises L-Phe to kPPV", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "extracellular region", "compartment_ids": [ "GO:0005576" ], "rhea_ids": [ "RHEA:61241" ], "source_url": "https://reactome.org/content/detail/R-HSA-2160492", "summary": "IL4I1:FAD oxidises L-Phe to kPPV is a Reactome release 97 curated reaction event. The source record connects L-Phe, H2O, O2 to kPPV, H2O2, NH4+ in extracellular region and cross-references RHEA:61241.", "equation": "L-Phe + H2O + O2 → kPPV + H2O2 + NH4+", "inputs": [ { "node_id": "R-ALL-351993", "reactome_id": "R-ALL-351993", "label": "L-Phe", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58095", "formula": "C9H11NO2", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-352327", "reactome_id": "R-ALL-352327", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2160466", "reactome_id": "R-ALL-2160466", "label": "kPPV", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18005", "formula": "C9H7O3", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-351629", "reactome_id": "R-ALL-351629", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-140912", "reactome_id": "R-ALL-140912", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-amino-acid oxidase activity of IL4I1:FAD [extracellular region]", "activity_id": "2160498", "ec_numbers": [ "1.4.3.2" ], "accessions": null } ], "citations": [ { "id": "PMID:17356132", "title": "Human IL4I1 is a secreted L-phenylalanine oxidase expressed by mature dendritic cells that inhibits T-lymphocyte proliferation", "journal": "Blood", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17356132/" } ], "x": 6521.333333333333, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2160851", "label": "HAS1,2,3 mediate the polymerization and export of HA", "schema_class": "Polymerisation", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "plasma membrane · cytosol", "compartment_ids": [ "GO:0005886", "GO:0005829" ], "rhea_ids": [ "RHEA:12529", "RHEA:20466" ], "source_url": "https://reactome.org/content/detail/R-HSA-2160851", "summary": "HAS1,2,3 mediate the polymerization and export of HA is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects UDP-GlcA, UDP-GlcNAc to H+, UDP, HA in plasma membrane · cytosol and cross-references RHEA:12529, RHEA:20466.", "equation": "UDP-GlcA + UDP-GlcNAc → 2 H+ + 2 UDP + HA", "inputs": [ { "node_id": "R-ALL-158601", "reactome_id": "R-ALL-158601", "label": "UDP-GlcA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58052", "formula": "C15H19N2O18P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-162756", "reactome_id": "R-ALL-162756", "label": "UDP-GlcNAc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57705", "formula": "C17H25N3O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2160848", "reactome_id": "R-ALL-2160848", "label": "HA", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "Polymer", "currency": false } ], "catalysts": [ { "label": "hyaluronan synthase activity of HAS1,2,3:Mg2+ [plasma membrane]", "activity_id": "2142876", "ec_numbers": [ "2.4.1.212" ], "accessions": null } ], "citations": [ { "id": "PMID:9083017", "title": "Molecular cloning and characterization of a cDNA encoding the third putative mammalian hyaluronan synthase", "journal": "J Biol Chem", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9083017/" }, { "id": "PMID:8798544", "title": "Functional cloning of the cDNA for a human hyaluronan synthase", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8798544/" }, { "id": "PMID:8798477", "title": "Molecular identification of a putative human hyaluronan synthase", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8798477/" }, { "id": "PMID:35355017", "title": "Structure, substrate recognition and initiation of hyaluronan synthase", "journal": "Nature", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/35355017/" }, { "id": "PMID:37769351", "title": "Hyaluronan synthases; mechanisms, myths, \u0026 mysteries of three types of unique bifunctional glycosyltransferases", "journal": "Glycobiology", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/37769351/" }, { "id": "PMID:22343360", "title": "The hyaluronan synthase catalyzes the synthesis and membrane translocation of hyaluronan", "journal": "J Mol Biol", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22343360/" } ], "x": 2660, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-2161549", "label": "PGH2 is reduced to PGF2a by AKR1C3", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:45314" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161549", "summary": "PGH2 is reduced to PGF2a by AKR1C3 is a Reactome release 97 curated reaction event. The source record connects PGH2, NADPH, H+ to PGF2a, NADP+ in cytosol and cross-references RHEA:45314.", "equation": "PGH2 + NADPH + H+ → PGF2a + NADP+", "inputs": [ { "node_id": "R-ALL-265283", "reactome_id": "R-ALL-265283", "label": "PGH2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57405", "formula": "C20H31O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-879535", "reactome_id": "R-ALL-879535", "label": "PGF2a", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57404", "formula": "C20H33O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "prostaglandin H2 endoperoxidase reductase activity of AKR1C3 [cytosol]", "activity_id": "2161558", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10622721", "title": "cDNA cloning, expression and characterization of human prostaglandin F synthase", "journal": "FEBS Lett", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10622721/" }, { "id": "PMID:14979715", "title": "Crystal structure of human prostaglandin F synthase (AKR1C3)", "journal": "Biochemistry", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/14979715/" }, { "id": "PMID:16475787", "title": "Prostaglandin F2alpha formation from prostaglandin H2 by prostaglandin F synthase (PGFS): crystal structure of PGFS containing bimatoprost", "journal": "Biochemistry", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16475787/" } ], "x": 5008.666666666667, "y": 2076.8333333333335, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161567", "label": "LTB4 is oxidised to 12-oxoLTB4 by PTGR1", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:50609" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161567", "summary": "LTB4 is oxidised to 12-oxoLTB4 by PTGR1 is a Reactome release 97 curated reaction event. The source record connects LTB4, NADP+ to 12-oxoLTB4, NADPH, H+ in cytosol and cross-references RHEA:50609.", "equation": "LTB4 + NADP+ → 12-oxoLTB4 + NADPH + H+", "inputs": [ { "node_id": "R-ALL-266056", "reactome_id": "R-ALL-266056", "label": "LTB4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57461", "formula": "C20H31O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2142810", "reactome_id": "R-ALL-2142810", "label": "12-oxoLTB4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:133309", "formula": "C20H29O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "leukotriene B4 12-hydroxy dehydrogenase activity of PTGR1 [cytosol]", "activity_id": "2161632", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:8576264", "title": "cDNA cloning, expression, and mutagenesis study of leukotriene B4 12-hydroxydehydrogenase", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8576264/" }, { "id": "PMID:8394361", "title": "Enzymatic inactivation of leukotriene B4 by a novel enzyme found in the porcine kidney. Purification and properties of leukotriene B4 12-hydroxydehydrogenase", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8394361/" }, { "id": "PMID:9461497", "title": "Purification, cDNA cloning and expression of 15-oxoprostaglandin 13-reductase from pig lung", "journal": "Biochem J", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9461497/" } ], "x": 4451.333333333333, "y": 2206.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161614", "label": "PGD2 is reduced to 11-epi-PGF2a by AKRIC3", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:45317" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161614", "summary": "PGD2 is reduced to 11-epi-PGF2a by AKRIC3 is a Reactome release 97 curated reaction event. The source record connects PGD2, H+, NADPH to 11epi-PGF2a, NADP+ in cytosol and cross-references RHEA:45317.", "equation": "PGD2 + H+ + NADPH → 11epi-PGF2a + NADP+", "inputs": [ { "node_id": "R-ALL-879629", "reactome_id": "R-ALL-879629", "label": "PGD2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57406", "formula": "C20H31O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2142710", "reactome_id": "R-ALL-2142710", "label": "11epi-PGF2a", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:85173", "formula": "C20H33O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "prostaglandin D2 11-ketoreductase activity of AKR1C3 [cytosol]", "activity_id": "2161608", "ec_numbers": [ "1.1.1.188" ], "accessions": null } ], "citations": [ { "id": "PMID:3862115", "title": "Transformation of prostaglandin D2 to 9 alpha, 11 beta-(15S)-trihydroxyprosta-(5Z,13E)-dien-1-oic acid (9 alpha, 11 beta-prostaglandin F2): a unique biologically active prostaglandin produced enzymatically in vivo in humans", "journal": "Proc Natl Acad Sci U S A", "year": 1985, "url": "https://pubmed.ncbi.nlm.nih.gov/3862115/" }, { "id": "PMID:15047184", "title": "Synthesis of prostaglandin F ethanolamide by prostaglandin F synthase and identification of Bimatoprost as a potent inhibitor of the enzyme: new enzyme assay method using LC/ESI/MS", "journal": "Arch Biochem Biophys", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15047184/" }, { "id": "PMID:20036328", "title": "Aldo-keto reductase 1C3 expression in MCF-7 cells reveals roles in steroid hormone and prostaglandin metabolism that may explain its over-expression in breast cancer", "journal": "J Steroid Biochem Mol Biol", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20036328/" } ], "x": 4730, "y": 2206.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161651", "label": "PGE2 is converted to PGF2a by CBR1", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24510" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161651", "summary": "PGE2 is converted to PGF2a by CBR1 is a Reactome release 97 curated reaction event. The source record connects PGE2, NADPH, H+ to PGF2a, NADP+ in cytosol and cross-references RHEA:24510.", "equation": "PGE2 + NADPH + H+ → PGF2a + NADP+", "inputs": [ { "node_id": "R-ALL-265287", "reactome_id": "R-ALL-265287", "label": "PGE2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:606564", "formula": "C20H31O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-879535", "reactome_id": "R-ALL-879535", "label": "PGF2a", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57404", "formula": "C20H33O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "prostaglandin E2 9-reductase activity of CBR1 [cytosol]", "activity_id": "2161655", "ec_numbers": [ "1.1.1.189" ], "accessions": null } ], "citations": [ { "id": "PMID:7005231", "title": "Purification and properties of an NADPH-dependent carbonyl reductase from human brain. Relationship to prostaglandin 9-ketoreductase and xenobiotic ketone reductase", "journal": "J Biol Chem", "year": 1981, "url": "https://pubmed.ncbi.nlm.nih.gov/7005231/" }, { "id": "PMID:18493841", "title": "Different functions between human monomeric carbonyl reductase 3 and carbonyl reductase 1", "journal": "Mol Cell Biochem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18493841/" } ], "x": 5008.666666666667, "y": 2206.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161732", "label": "TXB2 is converted to 11dh-TXB2 by TXDH", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "endoplasmic reticulum lumen", "compartment_ids": [ "GO:0005788" ], "rhea_ids": [ "RHEA:52313" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161732", "summary": "TXB2 is converted to 11dh-TXB2 by TXDH is a Reactome release 97 curated reaction event. The source record connects TXB2, NAD+ to 11dh-TXB2, NADH, H+ in endoplasmic reticulum lumen and cross-references RHEA:52313.", "equation": "TXB2 + NAD+ → 11dh-TXB2 + NADH + H+", "inputs": [ { "node_id": "R-ALL-443890", "reactome_id": "R-ALL-443890", "label": "TXB2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:90696", "formula": "C20H33O6", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-194653", "reactome_id": "R-ALL-194653", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2142828", "reactome_id": "R-ALL-2142828", "label": "11dh-TXB2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:136539", "formula": "C20H31O6", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-194697", "reactome_id": "R-ALL-194697", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-156540", "reactome_id": "R-ALL-156540", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "11-hydroxythromboxane B2 dehydrogenase activity of TXDH [endoplasmic reticulum lumen]", "activity_id": "2161726", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:3823488", "title": "Radioimmunoassay for 11-dehydro-TXB2: a method for monitoring thromboxane production in vivo", "journal": "Prostaglandins", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3823488/" }, { "id": "PMID:3461463", "title": "11-Dehydrothromboxane B2: a quantitative index of thromboxane A2 formation in the human circulation", "journal": "Proc Natl Acad Sci U S A", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3461463/" }, { "id": "PMID:8200461", "title": "11-Hydroxythromboxane B2 dehydrogenase is identical to cytosolic aldehyde dehydrogenase", "journal": "FEBS Lett", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8200461/" } ], "x": 4451.333333333333, "y": 2336.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161775", "label": "ALOX12 oxidises LTA4 to LXA4", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:48585" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161775", "summary": "ALOX12 oxidises LTA4 to LXA4 is a Reactome release 97 curated reaction event. The source record connects LTA4, O2, H2O to LXA4 in cytosol and cross-references RHEA:48585.", "equation": "2 LTA4 + O2 + 2 H2O → 2 LXA4", "inputs": [ { "node_id": "R-ALL-9032038", "reactome_id": "R-ALL-9032038", "label": "LTA4", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57463", "formula": "C20H29O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2161759", "reactome_id": "R-ALL-2161759", "label": "LXA4", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:67026", "formula": "C20H31O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen of ALOX12:Fe2+ [cytosol]", "activity_id": "2161800", "ec_numbers": [ "1.13.11" ], "accessions": null } ], "citations": [ { "id": "PMID:8250832", "title": "Lipoxin synthase activity of human platelet 12-lipoxygenase", "journal": "Biochem J", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8250832/" }, { "id": "PMID:2155925", "title": "Lipoxin formation during human neutrophil-platelet interactions. Evidence for the transformation of leukotriene A4 by platelet 12-lipoxygenase in vitro", "journal": "J Clin Invest", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2155925/" }, { "id": "PMID:3770188", "title": "Evidence for a 5(6)-epoxytetraene intermediate in the biosynthesis of lipoxins in human leukocytes. Conversion into lipoxin A by cytosolic epoxide hydrolase", "journal": "FEBS Lett", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3770188/" } ], "x": 5670, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161779", "label": "LXA4 is oxidised to 15k-LXA4 by HPGD", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:41573" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161779", "summary": "LXA4 is oxidised to 15k-LXA4 by HPGD is a Reactome release 97 curated reaction event. The source record connects LXA4, NAD+ to 15k-LXA4, NADH, H+ in cytosol and cross-references RHEA:41573.", "equation": "LXA4 + NAD+ → 15k-LXA4 + NADH + H+", "inputs": [ { "node_id": "R-ALL-2161759", "reactome_id": "R-ALL-2161759", "label": "LXA4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:67026", "formula": "C20H31O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2142761", "reactome_id": "R-ALL-2142761", "label": "15k-LXA4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:78311", "formula": "C20H29O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "15-hydroxyprostaglandin dehydrogenase (NAD+) activity of HPGD dimer [cytosol]", "activity_id": "2161623", "ec_numbers": [ "1.1.1.141" ], "accessions": null } ], "citations": [ { "id": "PMID:10837478", "title": "Oxidoreductases in lipoxin A4 metabolic inactivation: a novel role for 15-oxoprostaglandin 13-reductase/leukotriene B4 12-hydroxydehydrogenase in inflammation", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10837478/" } ], "x": 5670, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161789", "label": "15S-HETE is oxidised to 15-oxoETE by 15-HEDH", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23261", "RHEA:23265", "RHEA:23260", "RHEA:23264" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161789", "summary": "15S-HETE is oxidised to 15-oxoETE by 15-HEDH is a Reactome release 97 curated reaction event. The source record connects 15S-HETE, NAD(P)+ to 15-oxoETE, NAD(P)H, H+ in cytosol and cross-references RHEA:23261, RHEA:23265, RHEA:23260, RHEA:23264.", "equation": "15S-HETE + NAD(P)+ → 15-oxoETE + NAD(P)H + H+", "inputs": [ { "node_id": "R-ALL-2142707", "reactome_id": "R-ALL-2142707", "label": "15S-HETE", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57409", "formula": "C20H31O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-428218", "reactome_id": "R-ALL-428218", "label": "NAD(P)+", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2142747", "reactome_id": "R-ALL-2142747", "label": "15-oxoETE", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57410", "formula": "C20H29O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-428206", "reactome_id": "R-ALL-428206", "label": "NAD(P)H", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "15-hydroxyicosatetraenoate dehydrogenase activity of 15-HEDH [cytosol]", "activity_id": "2161741", "ec_numbers": [ "1.1.1.232" ], "accessions": null } ], "citations": [ { "id": "PMID:17662651", "title": "Expression of 15-lipoxygenase type-1 in human mast cells", "journal": "Biochim Biophys Acta", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17662651/" } ], "x": 311.33333333333337, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161791", "label": "15S-HpETE is reduced to 15S-HETE by GPX1/2/4", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:76696" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161791", "summary": "15S-HpETE is reduced to 15S-HETE by GPX1/2/4 is a Reactome release 97 curated reaction event. The source record connects 15S-HpETE, GSH to 15S-HETE, GSSG, H2O in cytosol and cross-references RHEA:76696.", "equation": "15S-HpETE + 2 GSH → 15S-HETE + GSSG + H2O", "inputs": [ { "node_id": "R-ALL-2142718", "reactome_id": "R-ALL-2142718", "label": "15S-HpETE", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57446", "formula": "C20H31O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29450", "reactome_id": "R-ALL-29450", "label": "GSH", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57925", "formula": "C10H16N3O6S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2142707", "reactome_id": "R-ALL-2142707", "label": "15S-HETE", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57409", "formula": "C20H31O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111745", "reactome_id": "R-ALL-111745", "label": "GSSG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58297", "formula": "C20H30N6O12S2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glutathione peroxidase activity of GPX1/2/4 [cytosol]", "activity_id": "2161937", "ec_numbers": [ "1.11.1.9" ], "accessions": null } ], "citations": [ { "id": "PMID:2501828", "title": "Role of glutathione and glutathione peroxidase in human platelet arachidonic acid metabolism", "journal": "Prostaglandins", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2501828/" } ], "x": 4730, "y": 2336.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161844", "label": "15k-LXA4 is reduced to dhk-LXA4 by PTGR1", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:41593" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161844", "summary": "15k-LXA4 is reduced to dhk-LXA4 by PTGR1 is a Reactome release 97 curated reaction event. The source record connects 15k-LXA4, H+, NADH to dhk-LXA4, NAD+ in cytosol and cross-references RHEA:41593.", "equation": "15k-LXA4 + H+ + NADH → dhk-LXA4 + NAD+", "inputs": [ { "node_id": "R-ALL-2142761", "reactome_id": "R-ALL-2142761", "label": "15k-LXA4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:78311", "formula": "C20H29O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2142690", "reactome_id": "R-ALL-2142690", "label": "dhk-LXA4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:78325", "formula": "C20H31O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "13-lipoxin reductase activity of PTGR1 [cytosol]", "activity_id": "2161822", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:8576264", "title": "cDNA cloning, expression, and mutagenesis study of leukotriene B4 12-hydroxydehydrogenase", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8576264/" }, { "id": "PMID:10837478", "title": "Oxidoreductases in lipoxin A4 metabolic inactivation: a novel role for 15-oxoprostaglandin 13-reductase/leukotriene B4 12-hydroxydehydrogenase in inflammation", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10837478/" } ], "x": 5948.666666666667, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2161946", "label": "5S-HpETE is reduced to 5S-HETE by GPX1/2/4", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:48621" ], "source_url": "https://reactome.org/content/detail/R-HSA-2161946", "summary": "5S-HpETE is reduced to 5S-HETE by GPX1/2/4 is a Reactome release 97 curated reaction event. The source record connects 5S-HpETE, GSH to 5S-HETE, GSSG, H2O in cytosol and cross-references RHEA:48621.", "equation": "5S-HpETE + 2 GSH → 5S-HETE + GSSG + H2O", "inputs": [ { "node_id": "R-ALL-266010", "reactome_id": "R-ALL-266010", "label": "5S-HpETE", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57450", "formula": "C20H31O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29450", "reactome_id": "R-ALL-29450", "label": "GSH", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57925", "formula": "C10H16N3O6S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2142733", "reactome_id": "R-ALL-2142733", "label": "5S-HETE", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:90632", "formula": "C20H31O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111745", "reactome_id": "R-ALL-111745", "label": "GSSG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58297", "formula": "C20H30N6O12S2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glutathione peroxidase activity of GPX1/2/4 [cytosol]", "activity_id": "2161908", "ec_numbers": [ "1.11.1.9" ], "accessions": null } ], "citations": [ { "id": "PMID:6816802", "title": "Role of glutathione peroxidase and hexose monophosphate shunt in the platelet lipoxygenase pathway", "journal": "J Biol Chem", "year": 1982, "url": "https://pubmed.ncbi.nlm.nih.gov/6816802/" }, { "id": "PMID:10549853", "title": "Cellular glutathione peroxidase as a predominant scavenger of hydroperoxyeicosatetraenoic acids in rabbit alveolar macrophages", "journal": "Biol Pharm Bull", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10549853/" }, { "id": "PMID:11115402", "title": "Evidence for the presence of phospholipid hydroperoxide glutathione peroxidase in human platelets: implications for its involvement in the regulatory network of the 12-lipoxygenase pathway of arachidonic acid metabolism", "journal": "Biochem J", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11115402/" }, { "id": "PMID:8428933", "title": "Expression, characterization, and tissue distribution of a new cellular selenium-dependent glutathione peroxidase, GSHPx-GI", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8428933/" } ], "x": 5008.666666666667, "y": 2336.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2162186", "label": "COQ3 methylates DeMQ10H2", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:44412", "RHEA:44413" ], "source_url": "https://reactome.org/content/detail/R-HSA-2162186", "summary": "COQ3 methylates DeMQ10H2 is a Reactome release 97 curated reaction event. The source record connects DeMQ10H2, AdoMet to CoQH2, AdoHcy, H+ in mitochondrial matrix and cross-references RHEA:44412, RHEA:44413.", "equation": "DeMQ10H2 + AdoMet → CoQH2 + AdoHcy + H+", "inputs": [ { "node_id": "R-ALL-2162212", "reactome_id": "R-ALL-2162212", "label": "DeMQ10H2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:64182", "formula": "C58H90O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162265", "reactome_id": "R-ALL-2162265", "label": "AdoMet", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59789", "formula": "C15H23N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2162198", "reactome_id": "R-ALL-2162198", "label": "CoQH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:64183", "formula": "C59H92O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162252", "reactome_id": "R-ALL-2162252", "label": "AdoHcy", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57856", "formula": "C14H20N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "3-demethylubiquinol 3-O-methyltransferase activity of COQ3:COQ4:COQ5:COQ6:COQ7:COQ9 [mitochondrial inner membrane]", "activity_id": "2162293", "ec_numbers": [ "2.1.1.64" ], "accessions": null } ], "citations": [ { "id": "PMID:10777520", "title": "Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10777520/" }, { "id": "PMID:26251450", "title": "Structural and biochemical studies reveal UbiG/Coq3 as a class of novel membrane-binding proteins", "journal": "Biochem J", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/26251450/" }, { "id": "PMID:38425362", "title": "In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis", "journal": "Nat Catal", "year": 2024, "url": "https://pubmed.ncbi.nlm.nih.gov/38425362/" } ], "x": 4730, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2162188", "label": "COQ5 methylates MDMQ10H2", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:44764", "RHEA:44765" ], "source_url": "https://reactome.org/content/detail/R-HSA-2162188", "summary": "COQ5 methylates MDMQ10H2 is a Reactome release 97 curated reaction event. The source record connects MDMQ10H2, AdoMet to DMQ10H2, AdoHcy, H+ in mitochondrial matrix and cross-references RHEA:44764, RHEA:44765.", "equation": "MDMQ10H2 + AdoMet → DMQ10H2 + AdoHcy + H+", "inputs": [ { "node_id": "R-ALL-2162205", "reactome_id": "R-ALL-2162205", "label": "MDMQ10H2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:64180", "formula": "C57H88O3", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162265", "reactome_id": "R-ALL-2162265", "label": "AdoMet", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59789", "formula": "C15H23N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2162204", "reactome_id": "R-ALL-2162204", "label": "DMQ10H2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:64181", "formula": "C58H90O3", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162252", "reactome_id": "R-ALL-2162252", "label": "AdoHcy", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57856", "formula": "C14H20N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "2-methoxy-6-polyprenyl-1,4-benzoquinol methyltransferase activity of COQ3:COQ4:COQ5:COQ6:COQ7:COQ9 [mitochondrial inner membrane]", "activity_id": "2167835", "ec_numbers": [ "2.1.1.201" ], "accessions": null } ], "citations": [ { "id": "PMID:9083049", "title": "Characterization of the COQ5 gene from Saccharomyces cerevisiae. Evidence for a C-methyltransferase in ubiquinone biosynthesis", "journal": "J Biol Chem", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9083049/" }, { "id": "PMID:25152161", "title": "Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis", "journal": "Biochim Biophys Acta", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/25152161/" }, { "id": "PMID:27155576", "title": "Disruption of the human COQ5-containing protein complex is associated with diminished coenzyme Q10 levels under two different conditions of mitochondrial energy deficiency", "journal": "Biochim Biophys Acta", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27155576/" }, { "id": "PMID:23354120", "title": "Detection of suppressed maturation of the human COQ5 protein in the mitochondria following mitochondrial uncoupling by an antibody recognizing both precursor and mature forms of COQ5", "journal": "Mitochondrion", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23354120/" }, { "id": "PMID:38425362", "title": "In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis", "journal": "Nat Catal", "year": 2024, "url": "https://pubmed.ncbi.nlm.nih.gov/38425362/" } ], "x": 5008.666666666667, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2162192", "label": "COQ2 ligates all-E-10PrP2 to PHB", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:44564", "RHEA:44565" ], "source_url": "https://reactome.org/content/detail/R-HSA-2162192", "summary": "COQ2 ligates all-E-10PrP2 to PHB is a Reactome release 97 curated reaction event. The source record connects PHB, all-E-10PrP2 to DHB, PPi in mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:44564, RHEA:44565.", "equation": "PHB + all-E-10PrP2 → DHB + PPi", "inputs": [ { "node_id": "R-ALL-2162213", "reactome_id": "R-ALL-2162213", "label": "PHB", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17879", "formula": "C7H5O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162214", "reactome_id": "R-ALL-2162214", "label": "all-E-10PrP2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:60721", "formula": "C50H81O7P2", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2162207", "reactome_id": "R-ALL-2162207", "label": "DHB", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:84503", "formula": "C57H85O3", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "4-hydroxybenzoate polyprenyltransferase activity of COQ2 [mitochondrial inner membrane]", "activity_id": "2162263", "ec_numbers": [ "2.5.1.39" ], "accessions": null } ], "citations": [ { "id": "PMID:15153069", "title": "Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ", "journal": "Biochem J", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15153069/" }, { "id": "PMID:17916383", "title": "Investigation of coenzyme Q biosynthesis in human fibroblast and HepG2 cells", "journal": "J Biochem Biophys Methods", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/17916383/" }, { "id": "PMID:17374725", "title": "Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis", "journal": "Hum Mol Genet", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17374725/" } ], "x": 4730, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2162193", "label": "COQ3 methylates DHDB", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:44492", "RHEA:44493" ], "source_url": "https://reactome.org/content/detail/R-HSA-2162193", "summary": "COQ3 methylates DHDB is a Reactome release 97 curated reaction event. The source record connects DHDB, AdoMet to MHDB, AdoHcy, H+ in mitochondrial matrix and cross-references RHEA:44492, RHEA:44493.", "equation": "DHDB + AdoMet → MHDB + AdoHcy + H+", "inputs": [ { "node_id": "R-ALL-2162209", "reactome_id": "R-ALL-2162209", "label": "DHDB", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62793", "formula": "C57H85O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162265", "reactome_id": "R-ALL-2162265", "label": "AdoMet", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59789", "formula": "C15H23N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2162215", "reactome_id": "R-ALL-2162215", "label": "MHDB", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62796", "formula": "C58H87O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162252", "reactome_id": "R-ALL-2162252", "label": "AdoHcy", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57856", "formula": "C14H20N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "polyprenyldihydroxybenzoate methyltransferase activity of COQ3:COQ4:COQ5:COQ6:COQ7:COQ9 [mitochondrial inner membrane]", "activity_id": "2162287", "ec_numbers": [ "2.1.1.114" ], "accessions": null } ], "citations": [ { "id": "PMID:10777520", "title": "Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10777520/" }, { "id": "PMID:26251450", "title": "Structural and biochemical studies reveal UbiG/Coq3 as a class of novel membrane-binding proteins", "journal": "Biochem J", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/26251450/" }, { "id": "PMID:38425362", "title": "In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis", "journal": "Nat Catal", "year": 2024, "url": "https://pubmed.ncbi.nlm.nih.gov/38425362/" } ], "x": 4451.333333333333, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2162253", "label": "PDSS1,2 ligates FPP to IPPP", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:27802", "RHEA:27803" ], "source_url": "https://reactome.org/content/detail/R-HSA-2162253", "summary": "PDSS1,2 ligates FPP to IPPP is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects FPP, IPPP to all-E-10PrP2, PPi in mitochondrial matrix and cross-references RHEA:27802, RHEA:27803.", "equation": "FPP + 7 IPPP → all-E-10PrP2 + 7 PPi", "inputs": [ { "node_id": "R-ALL-2162257", "reactome_id": "R-ALL-2162257", "label": "FPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:175763", "formula": "C15H25O7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162256", "reactome_id": "R-ALL-2162256", "label": "IPPP", "role": "input", "stoichiometry": 7, "chebi_id": "CHEBI:128769", "formula": "C5H9O7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-2162214", "reactome_id": "R-ALL-2162214", "label": "all-E-10PrP2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:60721", "formula": "C50H81O7P2", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 7, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "all-trans-decaprenyl-diphosphate synthase activity of PDSS1/2 tetramer [mitochondrial matrix]", "activity_id": "2162261", "ec_numbers": [ "2.5.1.91" ], "accessions": null } ], "citations": [ { "id": "PMID:16262699", "title": "Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans", "journal": "FEBS J", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16262699/" }, { "id": "PMID:17916383", "title": "Investigation of coenzyme Q biosynthesis in human fibroblast and HepG2 cells", "journal": "J Biochem Biophys Methods", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/17916383/" } ], "x": 5008.666666666667, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-2395512", "label": "FDXR transfers electrons to FDX1,2 (FDX1L)", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "iron-sulfur" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-2395512", "summary": "FDXR transfers electrons to FDX1,2 (FDX1L) is a Reactome release 97 curated reaction event. The source record connects FDXR:FADH2, FDX1,FDX2 (oxidized) to FDXR:FAD, H+, FDX1,FDX2 (reduced) in mitochondrial matrix.", "equation": "FDXR:FADH2 + 2 FDX1,FDX2 (oxidized) → FDXR:FAD + 2 H+ + 2 FDX1,FDX2 (reduced)", "inputs": [ { "node_id": "R-HSA-2395502", "reactome_id": "R-HSA-2395502", "label": "FDXR:FADH2", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-HSA-2408370", "reactome_id": "R-HSA-2408370", "label": "FDX1,FDX2 (oxidized)", "role": "input", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-HSA-2395508", "reactome_id": "R-HSA-2395508", "label": "FDXR:FAD", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-2408372", "reactome_id": "R-HSA-2408372", "label": "FDX1,FDX2 (reduced)", "role": "output", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "ferredoxin-NADP+ reductase activity of FDXR [mitochondrial matrix]", "activity_id": "9954152", "ec_numbers": [ "1.18.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:22101253", "title": "Both human ferredoxins 1 and 2 and ferredoxin reductase are important for iron-sulfur cluster biogenesis", "journal": "Biochim. Biophys. Acta", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22101253/" }, { "id": "PMID:20547883", "title": "Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe/S cluster biosynthesis", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20547883/" } ], "x": 5391.333333333333, "y": 3573.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-2395517", "label": "NADPH transfers electrons to FDXR", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "iron-sulfur" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-2395517", "summary": "NADPH transfers electrons to FDXR is a Reactome release 97 curated reaction event. The source record connects NADPH, FDXR:FAD, H+ to NADP+, FDXR:FADH2 in mitochondrial matrix.", "equation": "NADPH + FDXR:FAD + H+ → NADP+ + FDXR:FADH2", "inputs": [ { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-2395508", "reactome_id": "R-HSA-2395508", "label": "FDXR:FAD", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-2395502", "reactome_id": "R-HSA-2395502", "label": "FDXR:FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false } ], "catalysts": [ { "label": "ferredoxin-NADP+ reductase activity of FDXR [mitochondrial matrix]", "activity_id": "9954152", "ec_numbers": [ "1.18.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:1567230", "title": "Expression and characterization of human mitochondrial ferredoxin reductase in Escherichia coli", "journal": "Arch. Biochem. Biophys.", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1567230/" } ], "x": 5670, "y": 3379.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-266070", "label": "LTC4 is exported from the cytosol by ABCC1", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "arachidonate", "fatty-acid-metabolism" ], "compartment": "plasma membrane · cytosol · extracellular region", "compartment_ids": [ "GO:0005886", "GO:0005829", "GO:0005576" ], "rhea_ids": [ "RHEA:38963", "RHEA:38964" ], "source_url": "https://reactome.org/content/detail/R-HSA-266070", "summary": "LTC4 is exported from the cytosol by ABCC1 is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects LTC4, ATP, H2O to H+, Pi, LTC4 in plasma membrane · cytosol · extracellular region and cross-references RHEA:38963, RHEA:38964.", "equation": "LTC4 + ATP + H2O → H+ + Pi + LTC4 + ADP", "inputs": [ { "node_id": "R-ALL-266066", "reactome_id": "R-ALL-266066", "label": "LTC4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57973", "formula": "C30H45N3O9S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-266013", "reactome_id": "R-ALL-266013", "label": "LTC4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57973", "formula": "C30H45N3O9S", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ABC-type transporter activity of ABCC1 [plasma membrane]", "activity_id": "266058", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:2753893", "title": "The identification of a distinct export step following the biosynthesis of leukotriene C4 by human eosinophils", "journal": "J Biol Chem", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2753893/" }, { "id": "PMID:10064732", "title": "Characterization of a leukotriene C4 export mechanism in human platelets: possible involvement of multidrug resistance-associated protein 1", "journal": "J Lipid Res", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10064732/" } ], "x": 868.6666666666667, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-2995334", "label": "COX15 transforms heme O to heme A", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:63388" ], "source_url": "https://reactome.org/content/detail/R-HSA-2995334", "summary": "COX15 transforms heme O to heme A is a Reactome release 97 curated reaction event. The source record connects hydrogen acceptor, H2O, heme O to hydrogen donor, heme A in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:63388.", "equation": "2 hydrogen acceptor + H2O + heme O → 2 hydrogen donor + heme A", "inputs": [ { "node_id": "R-ALL-9757044", "reactome_id": "R-ALL-9757044", "label": "hydrogen acceptor", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:13193", "formula": "A", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9865393", "reactome_id": "R-ALL-9865393", "label": "heme O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:60530", "formula": "C49H56FeN4O5", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9757039", "reactome_id": "R-ALL-9757039", "label": "hydrogen donor", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:17499", "formula": "H2A", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9864492", "reactome_id": "R-ALL-9864492", "label": "heme A", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:61715", "formula": "C49H54FeN4O6", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "heme A synthase activity of COX15 [mitochondrial inner membrane]", "activity_id": "2995322", "ec_numbers": [ "1.17.99.9" ], "accessions": null } ], "citations": [ { "id": "PMID:12474143", "title": "Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy", "journal": "Am. J. Hum. Genet.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12474143/" }, { "id": "PMID:15863660", "title": "Novel mutations in COX15 in a long surviving Leigh syndrome patient with cytochrome c oxidase deficiency", "journal": "J. Med. Genet.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15863660/" }, { "id": "PMID:15235026", "title": "Functional and genetic studies demonstrate that mutation in the COX15 gene can cause Leigh syndrome", "journal": "J. Med. Genet.", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15235026/" }, { "id": "PMID:9878253", "title": "Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain", "journal": "Genomics", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9878253/" }, { "id": "PMID:34428995", "title": "Biosynthesis and trafficking of heme o and heme a : new structural insights and their implications for reaction mechanisms and prenylated heme transfer", "journal": "Crit Rev Biochem Mol Biol", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/34428995/" } ], "x": 6800, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-3322001", "label": "GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18550" ], "source_url": "https://reactome.org/content/detail/R-HSA-3322001", "summary": "GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1 is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, oligoGlc-GYG1:GYS1-a tetramer to UDP, polyGlc-GYG1:GYS1-a tetramer, H+ in cytosol and cross-references RHEA:18550.", "equation": "8 UDP-Glc + oligoGlc-GYG1:GYS1-a tetramer → 8 UDP + polyGlc-GYG1:GYS1-a tetramer + 8 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322035", "reactome_id": "R-HSA-3322035", "label": "oligoGlc-GYG1:GYS1-a tetramer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322054", "reactome_id": "R-HSA-3322054", "label": "polyGlc-GYG1:GYS1-a tetramer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "alpha-1,4-glucan glucosyltransferase (UDP-glucose donor) activity of oligoGlc-GYG1:GYS1-a tetramer [cytosol]", "activity_id": "3322023", "ec_numbers": [ "2.4.1.11" ], "accessions": null } ], "citations": [ { "id": "PMID:19699667", "title": "Identification of a novel mutation in GYS1 (muscle-specific glycogen synthase) resulting in sudden cardiac death, that is diagnosable from skin fibroblasts", "journal": "Mol. Genet. Metab.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19699667/" }, { "id": "PMID:22248338", "title": "Glycogen and its metabolism: some new developments and old themes", "journal": "Biochem. J.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22248338/" } ], "x": 1530, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-3322003", "label": "Autoglucosylation of GYG1 complexed with GYS1-b", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23361" ], "source_url": "https://reactome.org/content/detail/R-HSA-3322003", "summary": "Autoglucosylation of GYG1 complexed with GYS1-b is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, GYG1:GYS1-b tetramer to UDP, oligoGlc-GYG1:GYS1-b tetramer, H+ in cytosol and cross-references RHEA:23361.", "equation": "8 UDP-Glc + GYG1:GYS1-b tetramer → 8 UDP + oligoGlc-GYG1:GYS1-b tetramer + 8 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322062", "reactome_id": "R-HSA-3322062", "label": "GYG1:GYS1-b tetramer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322053", "reactome_id": "R-HSA-3322053", "label": "oligoGlc-GYG1:GYS1-b tetramer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycogenin glucosyltransferase activity of GYG1:GYS1-b tetramer [cytosol]", "activity_id": "3322038", "ec_numbers": [ "2.4.1.186" ], "accessions": null } ], "citations": [ { "id": "PMID:20357282", "title": "Glycogenin-1 deficiency and inactivated priming of glycogen synthesis", "journal": "N. Engl. J. Med.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20357282/" }, { "id": "PMID:22248338", "title": "Glycogen and its metabolism: some new developments and old themes", "journal": "Biochem. J.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22248338/" } ], "x": 1808.6666666666667, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-3322009", "label": "GYS2 catalyzes the polyglucosylation of oligoGlc-GYG2", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18550" ], "source_url": "https://reactome.org/content/detail/R-HSA-3322009", "summary": "GYS2 catalyzes the polyglucosylation of oligoGlc-GYG2 is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, oligoGlc-GYG2:GYS2-a tetramer to UDP, polyGlc-GYG2:GYS2-a tetramer, H+ in cytosol and cross-references RHEA:18550.", "equation": "8 UDP-Glc + oligoGlc-GYG2:GYS2-a tetramer → 8 UDP + polyGlc-GYG2:GYS2-a tetramer + 8 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3321999", "reactome_id": "R-HSA-3321999", "label": "oligoGlc-GYG2:GYS2-a tetramer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322056", "reactome_id": "R-HSA-3322056", "label": "polyGlc-GYG2:GYS2-a tetramer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "alpha-1,4-glucan glucosyltransferase (UDP-glucose donor) activity of oligoGlc-GYG2:GYS2-a tetramer [cytosol]", "activity_id": "3322006", "ec_numbers": [ "2.4.1.11" ], "accessions": null } ], "citations": [ { "id": "PMID:9691087", "title": "Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0", "journal": "J Clin Invest", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9691087/" }, { "id": "PMID:22248338", "title": "Glycogen and its metabolism: some new developments and old themes", "journal": "Biochem. J.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22248338/" } ], "x": 1251.3333333333333, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-3322014", "label": "Autoglucosylation of GYG2 complexed with GYS2-a", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23361" ], "source_url": "https://reactome.org/content/detail/R-HSA-3322014", "summary": "Autoglucosylation of GYG2 complexed with GYS2-a is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, GYG2:GYS2-a tetramer to UDP, oligoGlc-GYG2:GYS2-a tetramer, H+ in cytosol and cross-references RHEA:23361.", "equation": "8 UDP-Glc + GYG2:GYS2-a tetramer → 8 UDP + oligoGlc-GYG2:GYS2-a tetramer + 8 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322031", "reactome_id": "R-HSA-3322031", "label": "GYG2:GYS2-a tetramer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3321999", "reactome_id": "R-HSA-3321999", "label": "oligoGlc-GYG2:GYS2-a tetramer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycogenin glucosyltransferase activity of GYG2:GYS2-a tetramer [cytosol]", "activity_id": "3322010", "ec_numbers": [ "2.4.1.186" ], "accessions": null } ], "citations": [ { "id": "PMID:9346895", "title": "Glycogenin-2, a novel self-glucosylating protein involved in liver glycogen biosynthesis.", "journal": "J Biol Chem", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9346895/" }, { "id": "PMID:22248338", "title": "Glycogen and its metabolism: some new developments and old themes", "journal": "Biochem. 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The source record connects UDP-Glc, GYG2:GYS2-b tetramer to UDP, oligoGlc-GYG2:GYS2-b tetramer, H+ in cytosol and cross-references RHEA:23361.", "equation": "8 UDP-Glc + GYG2:GYS2-b tetramer → 8 UDP + oligoGlc-GYG2:GYS2-b tetramer + 8 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322065", "reactome_id": "R-HSA-3322065", "label": "GYG2:GYS2-b tetramer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322007", "reactome_id": "R-HSA-3322007", "label": "oligoGlc-GYG2:GYS2-b tetramer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycogenin glucosyltransferase activity of GYG2:GYS2-b tetramer [cytosol]", "activity_id": "3322066", "ec_numbers": [ "2.4.1.186" ], "accessions": null } ], "citations": [ { "id": "PMID:9346895", "title": "Glycogenin-2, a novel self-glucosylating protein involved in liver glycogen biosynthesis.", "journal": "J Biol Chem", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9346895/" }, { "id": "PMID:22248338", "title": "Glycogen and its metabolism: some new developments and old themes", "journal": "Biochem. 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The source record connects UDP-Glc, GYG1:GYS1-a tetramer to UDP, oligoGlc-GYG1:GYS1-a tetramer, H+ in cytosol and cross-references RHEA:23361.", "equation": "8 UDP-Glc + GYG1:GYS1-a tetramer → 8 UDP + oligoGlc-GYG1:GYS1-a tetramer + 8 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322050", "reactome_id": "R-HSA-3322050", "label": "GYG1:GYS1-a tetramer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322035", "reactome_id": "R-HSA-3322035", "label": "oligoGlc-GYG1:GYS1-a tetramer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycogenin glucosyltransferase activity of GYG1:GYS1-a tetramer [cytosol]", "activity_id": "3322020", "ec_numbers": [ "2.4.1.186" ], "accessions": null } ], "citations": [ { "id": "PMID:20357282", "title": "Glycogenin-1 deficiency and inactivated priming of glycogen synthesis", "journal": "N. Engl. J. Med.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20357282/" }, { "id": "PMID:22248338", "title": "Glycogen and its metabolism: some new developments and old themes", "journal": "Biochem. J.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22248338/" } ], "x": 1251.3333333333333, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-3322041", "label": "Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18550" ], "source_url": "https://reactome.org/content/detail/R-HSA-3322041", "summary": "Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1 is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, oligoGlc-GYG1:GYS1-b tetramer to UDP, polyGlc-GYG1:GYS1-b tetramer, H+ in cytosol and cross-references RHEA:18550.", "equation": "8 UDP-Glc + oligoGlc-GYG1:GYS1-b tetramer → 8 UDP + polyGlc-GYG1:GYS1-b tetramer + 8 H+", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322053", "reactome_id": "R-HSA-3322053", "label": "oligoGlc-GYG1:GYS1-b tetramer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-ALL-110096", "reactome_id": "R-ALL-110096", "label": "UDP", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-3322063", "reactome_id": "R-HSA-3322063", "label": "polyGlc-GYG1:GYS1-b tetramer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "alpha-1,4-glucan glucosyltransferase (UDP-glucose donor) activity of oligoGlc-GYG1:GYS1-b tetramer [cytosol]", "activity_id": "3322055", "ec_numbers": [ "2.4.1.11" ], "accessions": null } ], "citations": [ { "id": "PMID:19699667", "title": "Identification of a novel mutation in GYS1 (muscle-specific glycogen synthase) resulting in sudden cardiac death, that is diagnosable from skin fibroblasts", "journal": "Mol. Genet. Metab.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19699667/" }, { "id": "PMID:22248338", "title": "Glycogen and its metabolism: some new developments and old themes", "journal": "Biochem. J.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22248338/" } ], "x": 1530, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-352232", "label": "SLC7A5:SLC3A2 transports neutral amino acids from extracellular region to cytosol", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:73011", "RHEA:70947", "RHEA:72807", "RHEA:70943", "RHEA:68572", "RHEA:70719", "RHEA:27950", "RHEA:70939", "RHEA:29703" ], "source_url": "https://reactome.org/content/detail/R-HSA-352232", "summary": "SLC7A5:SLC3A2 transports neutral amino acids from extracellular region to cytosol is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects ligands of SLC7A5 to ligands of SLC7A5 in plasma membrane and cross-references RHEA:73011, RHEA:70947, RHEA:72807, RHEA:70943, RHEA:68572, RHEA:70719, RHEA:27950, RHEA:70939, RHEA:29703.", "equation": "ligands of SLC7A5 → ligands of SLC7A5", "inputs": [ { "node_id": "R-ALL-352229", "reactome_id": "R-ALL-352229", "label": "ligands of SLC7A5", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-352227", "reactome_id": "R-ALL-352227", "label": "ligands of SLC7A5", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "L-amino acid transmembrane transporter activity of SLC7A5:SLC3A2 [plasma membrane]", "activity_id": "352233", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10049700", "title": "Human LAT1, a subunit of system L amino acid transporter: molecular cloning and transport function", "journal": "Biochem Biophys Res Commun", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10049700/" }, { "id": "PMID:10391915", "title": "Identification of a membrane protein, LAT-2, that Co-expresses with 4F2 heavy chain, an L-type amino acid transport activity with broad specificity for small and large zwitterionic amino acids", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10391915/" }, { "id": "PMID:18262359", "title": "Functional characterization of tyrosine transport in fibroblast cells from healthy controls", "journal": "Neurosci Lett", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18262359/" } ], "x": 7461.333333333333, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-372342", "label": "MPC1:MPC2 imports PYR, H+ to mitochondrial matrix", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "mitochondrial intermembrane space · mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005758", "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:64720", "RHEA:64721" ], "source_url": "https://reactome.org/content/detail/R-HSA-372342", "summary": "MPC1:MPC2 imports PYR, H+ to mitochondrial matrix is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects H+, PYR to H+, PYR in mitochondrial intermembrane space · mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:64720, RHEA:64721.", "equation": "H+ + PYR → H+ + PYR", "inputs": [ { "node_id": "R-ALL-163953", "reactome_id": "R-ALL-163953", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9012375", "reactome_id": "R-ALL-9012375", "label": "PYR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113557", "reactome_id": "R-ALL-113557", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "pyruvate transmembrane transporter activity of MPC dimer [mitochondrial inner membrane]", "activity_id": "372338", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:22628558", "title": "A mitochondrial pyruvate carrier required for pyruvate uptake in yeast, Drosophila, and humans", "journal": "Science", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22628558/" }, { "id": "PMID:4822737", "title": "Specific inhibition of pyruvate transport in rat liver mitochondria and human erythrocytes by alpha-cyano-4-hydroxycinnamate", "journal": "Biochem. J.", "year": 1974, "url": "https://pubmed.ncbi.nlm.nih.gov/4822737/" }, { "id": "PMID:11945601", "title": "The transport of pyruvate in rat liver mitochondria", "journal": "FEBS Lett", "year": 1971, "url": "https://pubmed.ncbi.nlm.nih.gov/11945601/" }, { "id": "PMID:27317664", "title": "MPC1-like Is a Placental Mammal-specific Mitochondrial Pyruvate Carrier Subunit Expressed in Postmeiotic Male Germ Cells", "journal": "J Biol Chem", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27317664/" }, { "id": "PMID:33227948", "title": "Insights on the Quest for the Structure-Function Relationship of the Mitochondrial Pyruvate Carrier", "journal": "Biology (Basel)", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/33227948/" }, { "id": "PMID:32403431", "title": "Characteristic Analysis of Homo- and Heterodimeric Complexes of Human Mitochondrial Pyruvate Carrier Related to Metabolic Diseases", "journal": "Int J Mol Sci", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/32403431/" } ], "x": 4451.333333333333, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-372448", "label": "SLC25A12,13 exchange L-Glu and L-Asp", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "electron-transport", "malate-aspartate-shuttle" ], "compartment": "cytosol · mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005829", "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:70783" ], "source_url": "https://reactome.org/content/detail/R-HSA-372448", "summary": "SLC25A12,13 exchange L-Glu and L-Asp is a Reactome release 97 curated reaction event. The source record connects L-Asp, L-Glu, H+ to L-Asp, L-Glu, H+ in cytosol · mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:70783.", "equation": "L-Asp + L-Glu + H+ → L-Asp + L-Glu + H+", "inputs": [ { "node_id": "R-ALL-113553", "reactome_id": "R-ALL-113553", "label": "L-Asp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29448", "reactome_id": "R-ALL-29448", "label": "L-Asp", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acidic amino acid transmembrane transporter activity of SLC25A12,13 [mitochondrial inner membrane]", "activity_id": "372473", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:11566871", "title": "Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria", "journal": "EMBO J", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11566871/" }, { "id": "PMID:10642534", "title": "Characterization of a second member of the subfamily of calcium-binding mitochondrial carriers expressed in human non-excitable tissues", "journal": "Biochem J", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10642534/" }, { "id": "PMID:10369257", "title": "The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein", "journal": "Nat Genet", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10369257/" }, { "id": "PMID:12602510", "title": "Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency", "journal": "Metab Brain Dis", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12602510/" }, { "id": "PMID:36967723", "title": "Exogenous aralar/slc25a12 can replace citrin/slc25a13 as malate aspartate shuttle component in liver", "journal": "Mol Genet Metab Rep", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/36967723/" }, { "id": "PMID:32740958", "title": "Metabolic basis and treatment of citrin deficiency", "journal": "J Inherit Metab Dis", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/32740958/" } ], "x": 6521.333333333333, "y": 457.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-372819", "label": "PCK2 phosphorylates OA to yield PEP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:10389" ], "source_url": "https://reactome.org/content/detail/R-HSA-372819", "summary": "PCK2 phosphorylates OA to yield PEP is a Reactome release 97 curated reaction event. The source record connects OA, GTP to PEP, CO2, GDP in mitochondrial matrix and cross-references RHEA:10389.", "equation": "OA + GTP → PEP + CO2 + GDP", "inputs": [ { "node_id": "R-ALL-113587", "reactome_id": "R-ALL-113587", "label": "OA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113573", "reactome_id": "R-ALL-113573", "label": "GTP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:37565", "formula": "C10H12N5O14P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-372364", "reactome_id": "R-ALL-372364", "label": "PEP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58702", "formula": "C3H2O6P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113525", "reactome_id": "R-ALL-113525", "label": "GDP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58189", "formula": "C10H12N5O11P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoenolpyruvate carboxykinase (GTP) activity of PCK2 [mitochondrial matrix]", "activity_id": "372821", "ec_numbers": [ "4.1.1.32" ], "accessions": null } ], "citations": [ { "id": "PMID:9657976", "title": "Human mitochondrial phosphoenolpyruvate carboxykinase 2 gene. Structure, chromosomal localization and tissue-specific expression", "journal": "Biochem J", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9657976/" }, { "id": "PMID:8645161", "title": "Molecular cloning, sequencing and expression of the cDNA of the mitochondrial form of phosphoenolpyruvate carboxykinase from human liver", "journal": "Biochem J", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8645161/" } ], "x": 1808.6666666666667, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-376851", "label": "SLC25A11 exchanges 2-OG and MAL", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport", "malate-aspartate-shuttle" ], "compartment": "cytosol · mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005829", "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:71587", "RHEA:71589" ], "source_url": "https://reactome.org/content/detail/R-HSA-376851", "summary": "SLC25A11 exchanges 2-OG and MAL is a Reactome release 97 curated reaction event. The source record connects MAL, 2OG to MAL, 2OG in cytosol · mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:71587, RHEA:71589.", "equation": "MAL + 2OG → MAL + 2OG", "inputs": [ { "node_id": "R-ALL-198498", "reactome_id": "R-ALL-198498", "label": "MAL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113544", "reactome_id": "R-ALL-113544", "label": "MAL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "oxoglutarate:malate antiporter activity of SLC25A11 homodimer [mitochondrial inner membrane]", "activity_id": "198502", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:16920706", "title": "Porphyrin accumulation in mitochondria is mediated by 2-oxoglutarate carrier", "journal": "J Biol Chem", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16920706/" }, { "id": "PMID:8597574", "title": "The formation of a disulfide cross-link between the two subunits demonstrates the dimeric structure of the mitochondrial oxoglutarate carrier", "journal": "Biochim Biophys Acta", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8597574/" }, { "id": "PMID:4687397", "title": "Mechanism of the exchanges catalysed by the oxoglutarate translocator of rat-heart mitochondria. Kinetics of the external-product inhibition", "journal": "Eur J Biochem", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4687397/" }, { "id": "PMID:25637873", "title": "The mitochondrial dicarboxylate and 2-oxoglutarate carriers do not transport glutathione", "journal": "FEBS Lett", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25637873/" }, { "id": "PMID:29431636", "title": "Germline Mutations in the Mitochondrial 2-Oxoglutarate/Malate Carrier SLC25A11 Gene Confer a Predisposition to Metastatic Paragangliomas", "journal": "Cancer Res", "year": 2018, "url": "https://pubmed.ncbi.nlm.nih.gov/29431636/" } ], "x": 4730, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389540", "label": "isocitrate + NADP+ =\u003e 2-oxoglutarate + CO2 + NADPH + H+", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19630" ], "source_url": "https://reactome.org/content/detail/R-HSA-389540", "summary": "isocitrate + NADP+ =\u003e 2-oxoglutarate + CO2 + NADPH + H+ is a Reactome release 97 curated reaction event. The source record connects ISCIT, NADP+ to 2OG, CO2, NADPH in cytosol and cross-references RHEA:19630.", "equation": "ISCIT + NADP+ → 2OG + CO2 + NADPH + H+", "inputs": [ { "node_id": "R-ALL-389545", "reactome_id": "R-ALL-389545", "label": "ISCIT", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15562", "formula": "C6H5O7", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "isocitrate dehydrogenase (NADP+) activity of IDH1 dimer [cytosol]", "activity_id": "389538", "ec_numbers": [ "1.1.1.42" ], "accessions": null } ], "citations": [ { "id": "PMID:10521434", "title": "The human PICD gene encodes a cytoplasmic and peroxisomal NADP(+)-dependent isocitrate dehydrogenase", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10521434/" }, { "id": "PMID:15173171", "title": "Structures of human cytosolic NADP-dependent isocitrate dehydrogenase reveal a novel self-regulatory mechanism of activity", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15173171/" }, { "id": "PMID:11083877", "title": "Identification of the human mitochondrial oxodicarboxylate carrier. Bacterial expression, reconstitution, functional characterization, tissue distribution, and chromosomal location", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11083877/" } ], "x": 4730, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389550", "label": "isocitrate + NADP+ =\u003e 2-oxoglutarate + CO2 + NADPH + H+", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:19630" ], "source_url": "https://reactome.org/content/detail/R-HSA-389550", "summary": "isocitrate + NADP+ =\u003e 2-oxoglutarate + CO2 + NADPH + H+ is a Reactome release 97 curated reaction event. The source record connects ISCIT, NADP+ to 2OG, CO2, NADPH in peroxisomal matrix and cross-references RHEA:19630.", "equation": "ISCIT + NADP+ → 2OG + CO2 + NADPH + H+", "inputs": [ { "node_id": "R-ALL-389548", "reactome_id": "R-ALL-389548", "label": "ISCIT", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15562", "formula": "C6H5O7", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389556", "reactome_id": "R-ALL-389556", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-389537", "reactome_id": "R-ALL-389537", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389536", "reactome_id": "R-ALL-389536", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113602", "reactome_id": "R-ALL-113602", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193465", "reactome_id": "R-ALL-193465", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "isocitrate dehydrogenase (NADP+) activity of IDH1 dimer [peroxisomal matrix]", "activity_id": "389544", "ec_numbers": [ "1.1.1.42" ], "accessions": null } ], "citations": [ { "id": "PMID:15173171", "title": "Structures of human cytosolic NADP-dependent isocitrate dehydrogenase reveal a novel self-regulatory mechanism of activity", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15173171/" }, { "id": "PMID:10521434", "title": "The human PICD gene encodes a cytoplasmic and peroxisomal NADP(+)-dependent isocitrate dehydrogenase", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10521434/" } ], "x": 5008.666666666667, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389609", "label": "ALDH3A2-2 oxidizes pristanal to pristanate", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:44017" ], "source_url": "https://reactome.org/content/detail/R-HSA-389609", "summary": "ALDH3A2-2 oxidizes pristanal to pristanate is a Reactome release 97 curated reaction event. The source record connects NAD+, Pristanal, H2O to pristanate, NADH, H+ in peroxisomal matrix and cross-references RHEA:44017.", "equation": "NAD+ + Pristanal + H2O → pristanate + NADH + 2 H+", "inputs": [ { "node_id": "R-ALL-192307", "reactome_id": "R-ALL-192307", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-389636", "reactome_id": "R-ALL-389636", "label": "Pristanal", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:49189", "formula": "C19H38O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6811612", "reactome_id": "R-ALL-6811612", "label": "pristanate", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:77268", "formula": "C19H37O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-192305", "reactome_id": "R-ALL-192305", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193465", "reactome_id": "R-ALL-193465", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aldehyde dehydrogenase (NAD+) activity of ALDH3A2-2 dimer [peroxisomal membrane]", "activity_id": "6811622", "ec_numbers": [ "1.2.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:17510064", "title": "Dual subcellular localization in the endoplasmic reticulum and peroxisomes and a vital role in protecting against oxidative stress of fatty aldehyde dehydrogenase are achieved by alternative splicing", "journal": "J. Biol. Chem.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17510064/" }, { "id": "PMID:11341778", "title": "Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes", "journal": "Biochem Biophys Res Commun", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11341778/" }, { "id": "PMID:19965611", "title": "Monitoring of fatty aldehyde dehydrogenase by formation of pyrenedecanoic acid from pyrenedecanal", "journal": "J. Lipid Res.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/19965611/" }, { "id": "PMID:9133646", "title": "Human liver fatty aldehyde dehydrogenase: microsomal localization, purification, and biochemical characterization", "journal": "Biochim. Biophys. Acta", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9133646/" }, { "id": "PMID:11306053", "title": "Fatty aldehyde dehydrogenase: genomic structure, expression and mutation analysis in Sjögren-Larsson syndrome", "journal": "Chem. Biol. Interact.", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11306053/" } ], "x": 590, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389622", "label": "phytanate + CoA-SH + ATP =\u003e phytanoyl-CoA + AMP + pyrophosphate", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:21381" ], "source_url": "https://reactome.org/content/detail/R-HSA-389622", "summary": "phytanate + CoA-SH + ATP =\u003e phytanoyl-CoA + AMP + pyrophosphate is a Reactome release 97 curated reaction event. The source record connects ATP, CoA-SH, Phytanate to AMP, Phytanoyl-CoA, PPi in peroxisomal matrix and cross-references RHEA:21381.", "equation": "ATP + CoA-SH + Phytanate → AMP + Phytanoyl-CoA + PPi", "inputs": [ { "node_id": "R-ALL-389573", "reactome_id": "R-ALL-389573", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193514", "reactome_id": "R-ALL-193514", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-389596", "reactome_id": "R-ALL-389596", "label": "Phytanate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:37257", "formula": "C20H39O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-389620", "reactome_id": "R-ALL-389620", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-389615", "reactome_id": "R-ALL-389615", "label": "Phytanoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57391", "formula": "C41H70N7O17P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389593", "reactome_id": "R-ALL-389593", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phytanate-CoA ligase activity of SLC27A2 [peroxisomal membrane]", "activity_id": "389629", "ec_numbers": [ "6.2.1.24" ], "accessions": null } ], "citations": [ { "id": "PMID:10198260", "title": "Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism", "journal": "Biochem Biophys Res Commun", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10198260/" } ], "x": 868.6666666666667, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389632", "label": "pristanate + CoA-SH + ATP =\u003e pristanoyl-CoA + AMP + pyrophosphate", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:47265" ], "source_url": "https://reactome.org/content/detail/R-HSA-389632", "summary": "pristanate + CoA-SH + ATP =\u003e pristanoyl-CoA + AMP + pyrophosphate is a Reactome release 97 curated reaction event. The source record connects ATP, CoA-SH, pristanate to AMP, (2R) Pristanoyl-CoA, PPi in peroxisomal matrix and cross-references RHEA:47265.", "equation": "ATP + CoA-SH + pristanate → AMP + (2R) Pristanoyl-CoA + PPi", "inputs": [ { "node_id": "R-ALL-389573", "reactome_id": "R-ALL-389573", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193514", "reactome_id": "R-ALL-193514", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-6811612", "reactome_id": "R-ALL-6811612", "label": "pristanate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:77268", "formula": "C19H37O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-389620", "reactome_id": "R-ALL-389620", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-389569", "reactome_id": "R-ALL-389569", "label": "(2R) Pristanoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:77250", "formula": "C40H68N7O17P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389593", "reactome_id": "R-ALL-389593", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "pristanate-CoA ligase activity of SLC27A2 [peroxisomal membrane]", "activity_id": "389637", "ec_numbers": [ "6.2.1" ], "accessions": null } ], "citations": [ { "id": "PMID:10198260", "title": "Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism", "journal": "Biochem Biophys Res Commun", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10198260/" }, { "id": "PMID:9662422", "title": "Involvement of microsomal fatty aldehyde dehydrogenase in the alpha-oxidation of phytanic acid", "journal": "FEBS Lett", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9662422/" } ], "x": 311.33333333333337, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389639", "label": "phytanoyl-CoA + 2-oxoglutarate + O2 =\u003e 2-hydroxyphytanoyl-CoA + succinate + CO2", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:16066" ], "source_url": "https://reactome.org/content/detail/R-HSA-389639", "summary": "phytanoyl-CoA + 2-oxoglutarate + O2 =\u003e 2-hydroxyphytanoyl-CoA + succinate + CO2 is a Reactome release 97 curated reaction event. The source record connects Phytanoyl-CoA, 2OG, O2 to 3S2HPhy-CoA, SUCCA, CO2 in peroxisomal matrix and cross-references RHEA:16066.", "equation": "Phytanoyl-CoA + 2OG + O2 → 3S2HPhy-CoA + SUCCA + CO2", "inputs": [ { "node_id": "R-ALL-389615", "reactome_id": "R-ALL-389615", "label": "Phytanoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57391", "formula": "C41H70N7O17P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389537", "reactome_id": "R-ALL-389537", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-389594", "reactome_id": "R-ALL-389594", "label": "3S2HPhy-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57334", "formula": "C41H70N7O18P3S", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389583", "reactome_id": "R-ALL-389583", "label": "SUCCA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30031", "formula": "C4H4O4", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389536", "reactome_id": "R-ALL-389536", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phytanoyl-CoA dioxygenase activity of PHYH:Fe++ [peroxisomal matrix]", "activity_id": "389595", "ec_numbers": [ "1.14.11.18" ], "accessions": null } ], "citations": [ { "id": "PMID:16186124", "title": "Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16186124/" }, { "id": "PMID:11555634", "title": "Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease", "journal": "Hum Mol Genet", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11555634/" } ], "x": 590, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389684", "label": "glyoxylate + alanine =\u003e glycine + pyruvate [peroxisome]", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:24249" ], "source_url": "https://reactome.org/content/detail/R-HSA-389684", "summary": "glyoxylate + alanine =\u003e glycine + pyruvate [peroxisome] is a Reactome release 97 curated reaction event. The source record connects glyoxylate, L-Ala to Gly, PYR in peroxisomal matrix and cross-references RHEA:24249.", "equation": "glyoxylate + L-Ala → Gly + PYR", "inputs": [ { "node_id": "R-ALL-389678", "reactome_id": "R-ALL-389678", "label": "glyoxylate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36655", "formula": "C2HO3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389664", "reactome_id": "R-ALL-389664", "label": "L-Ala", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57972", "formula": "C3H7NO2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-193446", "reactome_id": "R-ALL-193446", "label": "Gly", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389680", "reactome_id": "R-ALL-389680", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-alanine:glyoxylate transaminase activity of AGXT dimer [peroxisomal matrix]", "activity_id": "389659", "ec_numbers": [ "2.6.1.44" ], "accessions": null } ], "citations": [ { "id": "PMID:15802217", "title": "Overexpression of human alanine:glyoxylate aminotransferase in Escherichia coli: renaturation from guanidine-HCl and affinity for pyridoxal phosphate co-factor", "journal": "Protein Expr Purif", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15802217/" }, { "id": "PMID:3416563", "title": "Further studies on the activity and subcellular distribution of alanine:glyoxylate aminotransferase in the livers of patients with primary hyperoxaluria type 1", "journal": "Clin Sci (Lond)", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3416563/" }, { "id": "PMID:1703535", "title": "Identification of mutations associated with peroxisome-to-mitochondrion mistargeting of alanine/glyoxylate aminotransferase in primary hyperoxaluria type 1", "journal": "J Cell Biol", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/1703535/" }, { "id": "PMID:2363689", "title": "Human peroxisomal L-alanine: glyoxylate aminotransferase. Evolutionary loss of a mitochondrial targeting signal by point mutation of the initiation codon", "journal": "Biochem J", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2363689/" }, { "id": "PMID:12899834", "title": "Crystal structure of alanine:glyoxylate aminotransferase and the relationship between genotype and enzymatic phenotype in primary hyperoxaluria type 1", "journal": "J Mol Biol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12899834/" } ], "x": 590, "y": 3313.444444444445, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389821", "label": "glycine + O2 =\u003e glyoxylate + H2O2 + NH4+", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:11533" ], "source_url": "https://reactome.org/content/detail/R-HSA-389821", "summary": "glycine + O2 =\u003e glyoxylate + H2O2 + NH4+ is a Reactome release 97 curated reaction event. The source record connects Gly, H2O, O2 to glyoxylate, H2O2, NH4+ in peroxisomal matrix and cross-references RHEA:11533.", "equation": "Gly + H2O + O2 → glyoxylate + H2O2 + NH4+", "inputs": [ { "node_id": "R-ALL-193446", "reactome_id": "R-ALL-193446", "label": "Gly", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-389678", "reactome_id": "R-ALL-389678", "label": "glyoxylate", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:36655", "formula": "C2HO3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113575", "reactome_id": "R-ALL-113575", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389843", "reactome_id": "R-ALL-389843", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "D-amino-acid oxidase activity of DAO dimer [peroxisomal matrix]", "activity_id": "389825", "ec_numbers": [ "1.4.3.3" ], "accessions": null } ], "citations": [ { "id": "PMID:17088322", "title": "Crystal structure of human D-amino acid oxidase: context-dependent variability of the backbone conformation of the VAAGL hydrophobic stretch located at the si-face of the flavin ring", "journal": "Protein Sci", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17088322/" }, { "id": "PMID:16616139", "title": "Characterization of human D-amino acid oxidase", "journal": "FEBS Lett", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16616139/" } ], "x": 311.33333333333337, "y": 3160.5555555555557, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389826", "label": "glyoxylate + NADPH + H+ =\u003e glycolate + NADP+", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:10994" ], "source_url": "https://reactome.org/content/detail/R-HSA-389826", "summary": "glyoxylate + NADPH + H+ =\u003e glycolate + NADP+ is a Reactome release 97 curated reaction event. The source record connects glyoxylate, NADPH, H+ to NADP+, glycolate in peroxisomal matrix and cross-references RHEA:10994.", "equation": "glyoxylate + NADPH + H+ → NADP+ + glycolate", "inputs": [ { "node_id": "R-ALL-389678", "reactome_id": "R-ALL-389678", "label": "glyoxylate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36655", "formula": "C2HO3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113602", "reactome_id": "R-ALL-113602", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-193465", "reactome_id": "R-ALL-193465", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-389556", "reactome_id": "R-ALL-389556", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-389822", "reactome_id": "R-ALL-389822", "label": "glycolate", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29805", "formula": "C2H3O3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glyoxylate reductase (NADPH) activity of GRHPR [peroxisomal matrix]", "activity_id": "389805", "ec_numbers": [ "1.1.1.79" ], "accessions": null } ], "citations": [ { "id": "PMID:10524214", "title": "Identification and expression of a cDNA for human hydroxypyruvate/glyoxylate reductase", "journal": "Biochim Biophys Acta", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10524214/" }, { "id": "PMID:10484776", "title": "The gene encoding hydroxypyruvate reductase (GRHPR) is mutated in patients with primary hyperoxaluria type II", "journal": "Hum Mol Genet", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10484776/" } ], "x": 311.33333333333337, "y": 3237, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389842", "label": "HAO1 tetramer oxidizes glycolate to glyoxylate", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:25312" ], "source_url": "https://reactome.org/content/detail/R-HSA-389842", "summary": "HAO1 tetramer oxidizes glycolate to glyoxylate is a Reactome release 97 curated reaction event. The source record connects glycolate, O2 to glyoxylate, H2O2 in peroxisomal matrix and cross-references RHEA:25312.", "equation": "glycolate + O2 → glyoxylate + H2O2", "inputs": [ { "node_id": "R-ALL-389822", "reactome_id": "R-ALL-389822", "label": "glycolate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29805", "formula": "C2H3O3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-389678", "reactome_id": "R-ALL-389678", "label": "glyoxylate", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:36655", "formula": "C2HO3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113575", "reactome_id": "R-ALL-113575", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "(S)-2-hydroxy-acid oxidase activity of HAO1 tetramer [peroxisomal matrix]", "activity_id": "9010884", "ec_numbers": [ "1.1.3.15" ], "accessions": null } ], "citations": [ { "id": "PMID:10978532", "title": "Identification and expression of a cDNA for human glycolate oxidase", "journal": "Biochim Biophys Acta", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10978532/" }, { "id": "PMID:18215067", "title": "Active site and loop 4 movements within human glycolate oxidase: implications for substrate specificity and drug design", "journal": "Biochemistry", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18215067/" }, { "id": "PMID:17669354", "title": "Purification and characterization of recombinant human liver glycolate oxidase", "journal": "Arch Biochem Biophys", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17669354/" }, { "id": "PMID:10777549", "title": "Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10777549/" } ], "x": 868.6666666666667, "y": 3313.444444444445, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-389862", "label": "Conversion of glyoxylate to oxalate", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:14838" ], "source_url": "https://reactome.org/content/detail/R-HSA-389862", "summary": "Conversion of glyoxylate to oxalate is a Reactome release 97 curated reaction event. The source record connects glyoxylate, H2O, O2 to OX, H2O2 in peroxisomal matrix and cross-references RHEA:14838.", "equation": "glyoxylate + H2O + O2 → OX + H2O2", "inputs": [ { "node_id": "R-ALL-389678", "reactome_id": "R-ALL-389678", "label": "glyoxylate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36655", "formula": "C2HO3", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-389819", "reactome_id": "R-ALL-389819", "label": "OX", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30623", "formula": "C2O4", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113575", "reactome_id": "R-ALL-113575", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glyoxylate oxidase activity of HAO1 tetramer [peroxisomal matrix]", "activity_id": "389855", "ec_numbers": [ "1.2.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:10978532", "title": "Identification and expression of a cDNA for human glycolate oxidase", "journal": "Biochim Biophys Acta", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10978532/" }, { "id": "PMID:18215067", "title": "Active site and loop 4 movements within human glycolate oxidase: implications for substrate specificity and drug design", "journal": "Biochemistry", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18215067/" }, { "id": "PMID:17669354", "title": "Purification and characterization of recombinant human liver glycolate oxidase", "journal": "Arch Biochem Biophys", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17669354/" }, { "id": "PMID:10777549", "title": "Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10777549/" } ], "x": 590, "y": 3237, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-390393", "label": "Peroxisomal uptake of very long-chain fatty acyl CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism" ], "compartment": "peroxisomal membrane", "compartment_ids": [ "GO:0005778" ], "rhea_ids": [ "RHEA:67073", "RHEA:67081" ], "source_url": "https://reactome.org/content/detail/R-HSA-390393", "summary": "Peroxisomal uptake of very long-chain fatty acyl CoA is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects VLCFA-CoA, H2O, ATP to VLCFA, ADP, CoA-SH in peroxisomal membrane and cross-references RHEA:67073, RHEA:67081.", "equation": "VLCFA-CoA + 2 H2O + ATP → VLCFA + ADP + CoA-SH + 2 H+", "inputs": [ { "node_id": "R-ALL-5695971", "reactome_id": "R-ALL-5695971", "label": "VLCFA-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:138261", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-9914044", "reactome_id": "R-ALL-9914044", "label": "VLCFA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58950", "formula": "CO2R", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ATPase-coupled transmembrane transporter activity of ABCD1 homodimer [peroxisomal membrane]", "activity_id": "390390", "ec_numbers": [ "7.2.2" ], "accessions": null } ], "citations": [ { "id": "PMID:21145416", "title": "Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation", "journal": "Biochim Biophys Acta", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21145416/" }, { "id": "PMID:33500543", "title": "Acyl-CoA thioesterase activity of peroxisomal ABC protein ABCD1 is required for the transport of very long-chain acyl-CoA into peroxisomes", "journal": "Sci Rep", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/33500543/" }, { "id": "PMID:22493507", "title": "Peroxisomal fatty acid uptake mechanism in Saccharomyces cerevisiae", "journal": "J Biol Chem", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22493507/" } ], "x": 311.33333333333337, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-427555", "label": "SLC26A1,2 cotransport SO4(2-), anion from extracellular region to cytosol", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:73215", "RHEA:72387", "RHEA:72275", "RHEA:75091" ], "source_url": "https://reactome.org/content/detail/R-HSA-427555", "summary": "SLC26A1,2 cotransport SO4(2-), anion from extracellular region to cytosol is a Reactome release 97 curated reaction event. The source record connects SO4(2-), SLC26A1,2 cargo to SO4(2-), SLC26A1,2 cargo in plasma membrane and cross-references RHEA:73215, RHEA:72387, RHEA:72275, RHEA:75091.", "equation": "SO4(2-) + SLC26A1,2 cargo → SO4(2-) + SLC26A1,2 cargo", "inputs": [ { "node_id": "R-ALL-427648", "reactome_id": "R-ALL-427648", "label": "SO4(2-)", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16189", "formula": "O4S", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9941480", "reactome_id": "R-ALL-9941480", "label": "SLC26A1,2 cargo", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-174375", "reactome_id": "R-ALL-174375", "label": "SO4(2-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16189", "formula": "O4S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9941466", "reactome_id": "R-ALL-9941466", "label": "SLC26A1,2 cargo", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "sulfate transmembrane transporter activity of SLC26A1,2 [plasma membrane]", "activity_id": "427619", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:12713736", "title": "Characterization of the human sulfate anion transporter (hsat-1) protein and gene (SAT1; SLC26A1)", "journal": "DNA Cell Biol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12713736/" }, { "id": "PMID:7923357", "title": "The diastrophic dysplasia gene encodes a novel sulfate transporter: positional cloning by fine-structure linkage disequilibrium mapping", "journal": "Cell", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7923357/" }, { "id": "PMID:23506885", "title": "The SLC26 gene family of anion transporters and channels", "journal": "Mol. Aspects Med.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23506885/" }, { "id": "PMID:8300633", "title": "Functional expression cloning of the canalicular sulfate transport system of rat hepatocytes", "journal": "J Biol Chem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8300633/" }, { "id": "PMID:12759755", "title": "The SLC26 gene family of multifunctional anion exchangers", "journal": "Pflugers Arch", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/12759755/" }, { "id": "PMID:11241838", "title": "Mutations in the diastrophic dysplasia sulfate transporter (DTDST) gene (SLC26A2): 22 novel mutations, mutation review, associated skeletal phenotypes, and diagnostic relevance", "journal": "Hum Mutat", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11241838/" } ], "x": 2381.3333333333335, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-428273", "label": "SPHK1 phosphorylates sphingoid", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:51496" ], "source_url": "https://reactome.org/content/detail/R-HSA-428273", "summary": "SPHK1 phosphorylates sphingoid is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects ATP, SPG to ADP, S1P, H+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:51496.", "equation": "ATP + SPG → ADP + S1P + H+", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-428682", "reactome_id": "R-ALL-428682", "label": "SPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:84410", "formula": "C3H9NO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-5279322", "reactome_id": "R-ALL-5279322", "label": "S1P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:76941", "formula": "C3H8NO5PR", "compartment": "cytoplasm", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "lipid kinase activity of SPHK1 [cytosol]", "activity_id": "428281", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10802064", "title": "Functional characterization of human sphingosine kinase-1", "journal": "FEBS Lett", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10802064/" }, { "id": "PMID:10947957", "title": "Human sphingosine kinase: purification, molecular cloning and characterization of the native and recombinant enzymes", "journal": "Biochem J", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10947957/" }, { "id": "PMID:28415564", "title": "Mammalian sphingosine kinase (SphK) isoenzymes and isoform expression: challenges for SphK as an oncotarget", "journal": "Oncotarget", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28415564/" }, { "id": "PMID:23602659", "title": "Molecular basis of sphingosine kinase 1 substrate recognition and catalysis", "journal": "Structure", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23602659/" }, { "id": "PMID:21864225", "title": "The compartmentalization and translocation of the sphingosine kinases: mechanisms and functions in cell signaling and sphingolipid metabolism", "journal": "Crit Rev Biochem Mol Biol", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21864225/" }, { "id": "PMID:33171624", "title": "Transcriptional Regulation of Sphingosine Kinase 1", "journal": "Cells", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/33171624/" } ], "x": 3321.3333333333335, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-428681", "label": "PXLP-SGPL1 cleaves sphingoid-1-phosphates", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:18594", "RHEA:33508" ], "source_url": "https://reactome.org/content/detail/R-HSA-428681", "summary": "PXLP-SGPL1 cleaves sphingoid-1-phosphates is a Reactome release 97 curated reaction event. The source record connects S1P,SPAP to PETA, HXAL,HD2NAL in cytosol · endoplasmic reticulum membrane and cross-references RHEA:18594, RHEA:33508.", "equation": "S1P,SPAP → PETA + HXAL,HD2NAL", "inputs": [ { "node_id": "R-ALL-9844328", "reactome_id": "R-ALL-9844328", "label": "S1P,SPAP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-428686", "reactome_id": "R-ALL-428686", "label": "PETA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17553", "formula": "C2H8NO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9844327", "reactome_id": "R-ALL-9844327", "label": "HXAL,HD2NAL", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "sphinganine-1-phosphate aldolase activity of PXLP-SGPL1 [endoplasmic reticulum membrane]", "activity_id": "428675", "ec_numbers": [ "4.1.2.27" ], "accessions": null } ], "citations": [ { "id": "PMID:11018465", "title": "Human sphingosine-1-phosphate lyase: cDNA cloning, functional expression studies and mapping to chromosome 10q22(1)", "journal": "Biochim Biophys Acta", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/11018465/" }, { "id": "PMID:18558101", "title": "Sphingosine-1-phosphate lyase in development and disease: sphingolipid metabolism takes flight", "journal": "Biochim Biophys Acta", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18558101/" }, { "id": "PMID:30090628", "title": "Nephrotic syndrome and adrenal insufficiency caused by a variant in SGPL1", "journal": "Clin Kidney J", "year": 2018, "url": "https://pubmed.ncbi.nlm.nih.gov/30090628/" }, { "id": "PMID:30635364", "title": "Fifty years of lyase and a moment of truth: sphingosine phosphate lyase from discovery to disease", "journal": "J Lipid Res", "year": 2019, "url": "https://pubmed.ncbi.nlm.nih.gov/30635364/" } ], "x": 3600, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-429730", "label": "PPM1L dephosphorylates multiphospho-CERT1-2", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:20630", "RHEA:47005" ], "source_url": "https://reactome.org/content/detail/R-HSA-429730", "summary": "PPM1L dephosphorylates multiphospho-CERT1-2 is a Reactome release 97 curated reaction event. The source record connects H2O, PPM1L:VAPA/B dimer, p-S,S132,T-CERT1-2 to CERT1-2:PPM1L:VAPA/B dimer, Pi in cytosol · endoplasmic reticulum membrane and cross-references RHEA:20630, RHEA:47005.", "equation": "3 H2O + PPM1L:VAPA/B dimer + p-S,S132,T-CERT1-2 → CERT1-2:PPM1L:VAPA/B dimer + 3 Pi", "inputs": [ { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 3, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-429703", "reactome_id": "R-HSA-429703", "label": "PPM1L:VAPA/B dimer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "endoplasmic reticulum membrane", "entity_kind": "Complex", "currency": false }, { "node_id": "R-HSA-429695", "reactome_id": "R-HSA-429695", "label": "p-S,S132,T-CERT1-2", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "outputs": [ { "node_id": "R-HSA-429672", "reactome_id": "R-HSA-429672", "label": "CERT1-2:PPM1L:VAPA/B dimer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "endoplasmic reticulum membrane", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 3, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "protein serine/threonine phosphatase activity of PPM1L:VAPA/B dimer [endoplasmic reticulum membrane]", "activity_id": "429724", "ec_numbers": [ "3.1.3.16" ], "accessions": null } ], "citations": [ { "id": "PMID:18165232", "title": "Protein phosphatase 2Cepsilon is an endoplasmic reticulum integral membrane protein that dephosphorylates the ceramide transport protein CERT to enhance its association with organelle membranes", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18165232/" }, { "id": "PMID:31254361", "title": "Structure, functions and regulation of CERT, a lipid-transfer protein for the delivery of ceramide at the ER-Golgi membrane contact sites", "journal": "FEBS Lett", "year": 2019, "url": "https://pubmed.ncbi.nlm.nih.gov/31254361/" } ], "x": 3321.3333333333335, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-429749", "label": "SLC5A8 (SMCT) co-transports monocarboxylates and Na+ from extracellular region to cytosol", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "plasma membrane · cytosol · extracellular region", "compartment_ids": [ "GO:0005886", "GO:0005829", "GO:0005576" ], "rhea_ids": [ "RHEA:75803", "RHEA:75791", "RHEA:75799", "RHEA:75807", "RHEA:27814", "RHEA:72935", "RHEA:75795", "RHEA:72947", "RHEA:72951", "RHEA:72939", "RHEA:72943", "RHEA:72955" ], "source_url": "https://reactome.org/content/detail/R-HSA-429749", "summary": "SLC5A8 (SMCT) co-transports monocarboxylates and Na+ from extracellular region to cytosol is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects monocarboxylates transported by SLC5A8, Na+ to monocarboxylates transported by SLC5A8, Na+ in plasma membrane · cytosol · extracellular region and cross-references RHEA:75803, RHEA:75791, RHEA:75799, RHEA:75807, RHEA:27814, RHEA:72935, RHEA:75795, RHEA:72947, RHEA:72951, RHEA:72939, RHEA:72943, RHEA:72955.", "equation": "monocarboxylates transported by SLC5A8 + 2 Na+ → monocarboxylates transported by SLC5A8 + 2 Na+", "inputs": [ { "node_id": "R-ALL-429739", "reactome_id": "R-ALL-429739", "label": "monocarboxylates transported by SLC5A8", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-74113", "reactome_id": "R-ALL-74113", "label": "Na+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:29101", "formula": "Na", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-429748", "reactome_id": "R-ALL-429748", "label": "monocarboxylates transported by SLC5A8", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-83910", "reactome_id": "R-ALL-83910", "label": "Na+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:29101", "formula": "Na", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "monocarboxylic acid transmembrane transporter activity of SLC5A8 [plasma membrane]", "activity_id": "429740", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:15090606", "title": "The human tumour suppressor gene SLC5A8 expresses a Na+-monocarboxylate cotransporter", "journal": "J Physiol", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15090606/" }, { "id": "PMID:33353981", "title": "NAD + metabolism and its roles in cellular processes during ageing", "journal": "Nat Rev Mol Cell Biol", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/33353981/" }, { "id": "PMID:14966140", "title": "Functional identification of SLC5A8, a tumor suppressor down-regulated in colon cancer, as a Na(+)-coupled transporter for short-chain fatty acids", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/14966140/" } ], "x": 5391.333333333333, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-450971", "label": "NNT dimer transfers proton from NADPH to NAD+", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrion", "compartment_ids": [ "GO:0005739" ], "rhea_ids": [ "RHEA:47992" ], "source_url": "https://reactome.org/content/detail/R-HSA-450971", "summary": "NNT dimer transfers proton from NADPH to NAD+ is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects NAD+, NADPH, H+ to H+, NADH, NADP+ in mitochondrion and cross-references RHEA:47992.", "equation": "NAD+ + NADPH + H+ → H+ + NADH + NADP+", "inputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-163953", "reactome_id": "R-ALL-163953", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "NAD(P)+ transhydrogenase (Si-specific) activity of NNT dimer [mitochondrial inner membrane]", "activity_id": "450969", "ec_numbers": [ "1.6.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:10673423", "title": "The high-resolution structure of the NADP(H)-binding component (dIII) of proton-translocating transhydrogenase from human heart mitochondria", "journal": "Structure", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10673423/" }, { "id": "PMID:2005110", "title": "Mitochondrial energy-linked nicotinamide nucleotide transhydrogenase. Membrane topography of the bovine enzyme", "journal": "J Biol Chem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/2005110/" }, { "id": "PMID:34155914", "title": "Mitochondrial NAD(P) + Transhydrogenase: From Molecular Features to Physiology and Disease", "journal": "Antioxid Redox Signal", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/34155914/" }, { "id": "PMID:8616157", "title": "The cDNA sequence of proton-pumping nicotinamide nucleotide transhydrogenase from man and mouse", "journal": "Biochim Biophys Acta", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8616157/" }, { "id": "PMID:31462775", "title": "Structure and mechanism of mitochondrial proton-translocating transhydrogenase", "journal": "Nature", "year": 2019, "url": "https://pubmed.ncbi.nlm.nih.gov/31462775/" } ], "x": 5670, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-450984", "label": "IDH2 dimer decarboxylates isocitrate", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:19630", "RHEA:19629" ], "source_url": "https://reactome.org/content/detail/R-HSA-450984", "summary": "IDH2 dimer decarboxylates isocitrate is a Reactome release 97 curated reaction event. The source record connects ISCIT, NADP+ to 2OG, CO2, NADPH in mitochondrial matrix and cross-references RHEA:19630, RHEA:19629.", "equation": "ISCIT + NADP+ → 2OG + CO2 + NADPH", "inputs": [ { "node_id": "R-ALL-29938", "reactome_id": "R-ALL-29938", "label": "ISCIT", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15562", "formula": "C6H5O7", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "isocitrate dehydrogenase (NADP+) activity of IDH2 dimer [mitochondrial matrix]", "activity_id": "450976", "ec_numbers": [ "1.1.1.42" ], "accessions": null } ], "citations": [ { "id": "PMID:12207025", "title": "Crystal structure of porcine mitochondrial NADP+-dependent isocitrate dehydrogenase complexed with Mn2+ and isocitrate. Insights into the enzyme mechanism", "journal": "J Biol Chem", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12207025/" }, { "id": "PMID:18806796", "title": "Insights from retinitis pigmentosa into the roles of isocitrate dehydrogenases in the Krebs cycle", "journal": "Nat Genet", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18806796/" }, { "id": "PMID:14832224", "title": "Di- and triphosphopyridine nucleotide isocitric dehydrogenases in yeast", "journal": "J Biol Chem", "year": 1951, "url": "https://pubmed.ncbi.nlm.nih.gov/14832224/" }, { "id": "PMID:18914071", "title": "Biosynthesis of tricarboxylic acids by carbon dioxide fixation; enzymatic mechanisms", "journal": "J Biol Chem", "year": 1948, "url": "https://pubmed.ncbi.nlm.nih.gov/18914071/" }, { "id": "PMID:11278619", "title": "Control of mitochondrial redox balance and cellular defense against oxidative damage by mitochondrial NADP+-dependent isocitrate dehydrogenase", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11278619/" }, { "id": "PMID:20847235", "title": "IDH2 mutations in patients with D-2-hydroxyglutaric aciduria", "journal": "Science", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20847235/" } ], "x": 5948.666666666667, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-452036", "label": "arginine + H2O =\u003e ornithine + urea [ARG2]", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:20570" ], "source_url": "https://reactome.org/content/detail/R-HSA-452036", "summary": "arginine + H2O =\u003e ornithine + urea [ARG2] is a Reactome release 97 curated reaction event. The source record connects L-Arg, H2O to L-Orn, Urea in mitochondrial matrix and cross-references RHEA:20570.", "equation": "L-Arg + H2O → L-Orn + Urea", "inputs": [ { "node_id": "R-ALL-374064", "reactome_id": "R-ALL-374064", "label": "L-Arg", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:32682", "formula": "C6H15N4O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113585", "reactome_id": "R-ALL-113585", "label": "L-Orn", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:46911", "formula": "C5H13N2O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-374061", "reactome_id": "R-ALL-374061", "label": "Urea", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16199", "formula": "CH4N2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "arginase activity of ARG2 trimer [mitochondrial matrix]", "activity_id": "452020", "ec_numbers": [ "3.5.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:12859189", "title": "Human arginase II: crystal structure and physiological role in male and female sexual arousal", "journal": "Biochemistry", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12859189/" }, { "id": "PMID:8898077", "title": "Molecular cloning of cDNA for nonhepatic mitochondrial arginase (arginase II) and comparison of its induction with nitric oxide synthase in a murine macrophage-like cell line", "journal": "FEBS Lett", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8898077/" }, { "id": "PMID:9686347", "title": "The human arginases and arginase deficiency", "journal": "J Inherit Metab Dis", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9686347/" } ], "x": 1530, "y": 3237, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-453337", "label": "glycogen phosphorylase (PYGB) dimer b + 2 ATP =\u003e glycogen phosphorylase (PYGB) dimer a + 2 ADP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17990" ], "source_url": "https://reactome.org/content/detail/R-HSA-453337", "summary": "glycogen phosphorylase (PYGB) dimer b + 2 ATP =\u003e glycogen phosphorylase (PYGB) dimer a + 2 ADP is a Reactome release 97 curated reaction event. The source record connects PYGB dimer b form, ATP to PYGB dimer a form, ADP, H+ in cytosol and cross-references RHEA:17990.", "equation": "PYGB dimer b form + 2 ATP → PYGB dimer a form + 2 ADP + 2 H+", "inputs": [ { "node_id": "R-HSA-71863", "reactome_id": "R-HSA-71863", "label": "PYGB dimer b form", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-HSA-71869", "reactome_id": "R-HSA-71869", "label": "PYGB dimer a form", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphorylase kinase activity of phosphorylase kinase complex (PHKM) [cytosol]", "activity_id": "71521", "ec_numbers": [ "2.7.11.19" ], "accessions": null } ], "citations": [ { "id": "PMID:2667896", "title": "The family of glycogen phosphorylases: structure and function", "journal": "Crit Rev Biochem Mol Biol", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2667896/" } ], "x": 1808.6666666666667, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-482619", "label": "(d)NDP + ATP (d)NTP + ADP (NME1,2,3)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:27687", "RHEA:28583", "RHEA:25238", "RHEA:25099", "RHEA:27675", "RHEA:27691", "RHEA:27679", "RHEA:27683" ], "source_url": "https://reactome.org/content/detail/R-HSA-482619", "summary": "(d)NDP + ATP (d)NTP + ADP (NME1,2,3) is a Reactome release 97 curated reaction event. The source record connects (d)NDP, ATP to (d)NTP, ADP in cytosol and cross-references RHEA:27687, RHEA:28583, RHEA:25238, RHEA:25099, RHEA:27675, RHEA:27691, RHEA:27679, RHEA:27683.", "equation": "(d)NDP + ATP → (d)NTP + ADP", "inputs": [ { "node_id": "R-ALL-482627", "reactome_id": "R-ALL-482627", "label": "(d)NDP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-482625", "reactome_id": "R-ALL-482625", "label": "(d)NTP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside diphosphate kinase activity of NME1,2 hexamers [cytosol]", "activity_id": "482613", "ec_numbers": [ "2.7.4.6" ], "accessions": null } ], "citations": [ { "id": "PMID:13211603", "title": "Enzymatic phosphorylation of nucleoside diphosphates", "journal": "J Biol Chem", "year": 1954, "url": "https://pubmed.ncbi.nlm.nih.gov/13211603/" }, { "id": "PMID:12972261", "title": "Nucleotide binding to nucleoside diphosphate kinases: X-ray structure of human NDPK-A in complex with ADP and comparison to protein kinases", "journal": "J Mol Biol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12972261/" }, { "id": "PMID:11277919", "title": "Structural and catalytic properties and homology modelling of the human nucleoside diphosphate kinase C, product of the DRnm23 gene", "journal": "Eur J Biochem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11277919/" }, { "id": "PMID:1851158", "title": "Nucleoside diphosphate kinase from human erythrocytes. Structural characterization of the two polypeptide chains responsible for heterogeneity of the hexameric enzyme", "journal": "J Biol Chem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1851158/" }, { "id": "PMID:9488696", "title": "Substrate specificity of human nucleoside-diphosphate kinase revealed by transient kinetic analysis", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9488696/" } ], "x": 3321.3333333333335, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-482621", "label": "(d)NTP + ADP (d)NDP + ATP (NME1,2,3)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:27688", "RHEA:28584", "RHEA:25239", "RHEA:25100", "RHEA:27676", "RHEA:27692", "RHEA:27680", "RHEA:27684" ], "source_url": "https://reactome.org/content/detail/R-HSA-482621", "summary": "(d)NTP + ADP (d)NDP + ATP (NME1,2,3) is a Reactome release 97 curated reaction event. The source record connects (d)NTP, ADP to (d)NDP, ATP in cytosol and cross-references RHEA:27688, RHEA:28584, RHEA:25239, RHEA:25100, RHEA:27676, RHEA:27692, RHEA:27680, RHEA:27684.", "equation": "(d)NTP + ADP → (d)NDP + ATP", "inputs": [ { "node_id": "R-ALL-482625", "reactome_id": "R-ALL-482625", "label": "(d)NTP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-482627", "reactome_id": "R-ALL-482627", "label": "(d)NDP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside diphosphate kinase activity of NME1,2 hexamers [cytosol]", "activity_id": "482613", "ec_numbers": [ "2.7.4.6" ], "accessions": null } ], "citations": [ { "id": "PMID:13211603", "title": "Enzymatic phosphorylation of nucleoside diphosphates", "journal": "J Biol Chem", "year": 1954, "url": "https://pubmed.ncbi.nlm.nih.gov/13211603/" }, { "id": "PMID:12972261", "title": "Nucleotide binding to nucleoside diphosphate kinases: X-ray structure of human NDPK-A in complex with ADP and comparison to protein kinases", "journal": "J Mol Biol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12972261/" }, { "id": "PMID:11277919", "title": "Structural and catalytic properties and homology modelling of the human nucleoside diphosphate kinase C, product of the DRnm23 gene", "journal": "Eur J Biochem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11277919/" }, { "id": "PMID:1851158", "title": "Nucleoside diphosphate kinase from human erythrocytes. Structural characterization of the two polypeptide chains responsible for heterogeneity of the hexameric enzyme", "journal": "J Biol Chem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1851158/" }, { "id": "PMID:9488696", "title": "Substrate specificity of human nucleoside-diphosphate kinase revealed by transient kinetic analysis", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9488696/" } ], "x": 3600, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-482804", "label": "(d)NDP + ATP (d)NTP + ADP (NME4)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:27675", "RHEA:27687", "RHEA:25238", "RHEA:25099", "RHEA:27691", "RHEA:27679", "RHEA:27683", "RHEA:28583" ], "source_url": "https://reactome.org/content/detail/R-HSA-482804", "summary": "(d)NDP + ATP (d)NTP + ADP (NME4) is a Reactome release 97 curated reaction event. The source record connects (d)NDP, ATP to (d)NTP, ADP in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:27675, RHEA:27687, RHEA:25238, RHEA:25099, RHEA:27691, RHEA:27679, RHEA:27683, RHEA:28583.", "equation": "(d)NDP + ATP → (d)NTP + ADP", "inputs": [ { "node_id": "R-ALL-482803", "reactome_id": "R-ALL-482803", "label": "(d)NDP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-482807", "reactome_id": "R-ALL-482807", "label": "(d)NTP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside diphosphate kinase activity of NME4 hexamer [mitochondrial inner membrane]", "activity_id": "110637", "ec_numbers": [ "2.7.4.6" ], "accessions": null } ], "citations": [ { "id": "PMID:10799505", "title": "The human nm23-H4 gene product is a mitochondrial nucleoside diphosphate kinase", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10799505/" }, { "id": "PMID:9488696", "title": "Substrate specificity of human nucleoside-diphosphate kinase revealed by transient kinetic analysis", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9488696/" }, { "id": "PMID:18635542", "title": "The nucleoside diphosphate kinase D (NM23-H4) binds the inner mitochondrial membrane with high affinity to cardiolipin and couples nucleotide transfer with respiration", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18635542/" } ], "x": 3878.666666666667, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-482812", "label": "(d)NTP + ADP (d)NDP + ATP (NME4)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:27676", "RHEA:27688", "RHEA:25239", "RHEA:25100", "RHEA:27692", "RHEA:27680", "RHEA:27684", "RHEA:28584" ], "source_url": "https://reactome.org/content/detail/R-HSA-482812", "summary": "(d)NTP + ADP (d)NDP + ATP (NME4) is a Reactome release 97 curated reaction event. The source record connects (d)NTP, ADP to (d)NDP, ATP in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:27676, RHEA:27688, RHEA:25239, RHEA:25100, RHEA:27692, RHEA:27680, RHEA:27684, RHEA:28584.", "equation": "(d)NTP + ADP → (d)NDP + ATP", "inputs": [ { "node_id": "R-ALL-482807", "reactome_id": "R-ALL-482807", "label": "(d)NTP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-482803", "reactome_id": "R-ALL-482803", "label": "(d)NDP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside diphosphate kinase activity of NME4 hexamer [mitochondrial inner membrane]", "activity_id": "110637", "ec_numbers": [ "2.7.4.6" ], "accessions": null } ], "citations": [ { "id": "PMID:10799505", "title": "The human nm23-H4 gene product is a mitochondrial nucleoside diphosphate kinase", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10799505/" }, { "id": "PMID:9488696", "title": "Substrate specificity of human nucleoside-diphosphate kinase revealed by transient kinetic analysis", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9488696/" }, { "id": "PMID:18635542", "title": "The nucleoside diphosphate kinase D (NM23-H4) binds the inner mitochondrial membrane with high affinity to cardiolipin and couples nucleotide transfer with respiration", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18635542/" } ], "x": 3321.3333333333335, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-508040", "label": "glutamate + ATP + NADPH + H+ =\u003e L-glutamate gamma-semialdehyde + NADP+ + ADP + orthophosphate [P5CS]", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:33208" ], "source_url": "https://reactome.org/content/detail/R-HSA-508040", "summary": "glutamate + ATP + NADPH + H+ =\u003e L-glutamate gamma-semialdehyde + NADP+ + ADP + orthophosphate [P5CS] is a Reactome release 97 curated reaction event. The source record connects L-Glu, ATP, NADPH to L-Glu5S, NADP+, ADP in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:33208.", "equation": "L-Glu + ATP + NADPH + H+ → L-Glu5S + NADP+ + ADP + Pi", "inputs": [ { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31339", "reactome_id": "R-ALL-31339", "label": "L-Glu5S", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58066", "formula": "C5H9NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "delta1-pyrroline-5-carboxylate synthetase activity of P5CS dimers [mitochondrial inner membrane]", "activity_id": "508035", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10037775", "title": "Molecular enzymology of mammalian Delta1-pyrroline-5-carboxylate synthase. Alternative splice donor utilization generates isoforms with different sensitivity to ornithine inhibition", "journal": "J Biol Chem", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10037775/" } ], "x": 7461.333333333333, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-508179", "label": "a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate leu, ile, or val + alpha-ketoglutarate [BCAT2]", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:24815", "RHEA:18323", "RHEA:24803" ], "source_url": "https://reactome.org/content/detail/R-HSA-508179", "summary": "a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate leu, ile, or val + alpha-ketoglutarate [BCAT2] is a Reactome release 97 curated reaction event. The source record connects KIC, KMVA, KIV, L-Glu to 2OG, Leu, Ile, Val in mitochondrial matrix and cross-references RHEA:24815, RHEA:18323, RHEA:24803.", "equation": "KIC, KMVA, KIV + L-Glu → 2OG + Leu, Ile, Val", "inputs": [ { "node_id": "R-ALL-508181", "reactome_id": "R-ALL-508181", "label": "KIC, KMVA, KIV", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-508190", "reactome_id": "R-ALL-508190", "label": "Leu, Ile, Val", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "branched-chain-amino-acid:2-oxoglutarate transaminase activity of BCAT2 dimer [mitochondrial matrix]", "activity_id": "70711", "ec_numbers": [ "2.6.1.42" ], "accessions": null } ], "citations": [ { "id": "PMID:9165094", "title": "Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm)", "journal": "Biochim Biophys Acta", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9165094/" }, { "id": "PMID:11264579", "title": "The structure of human mitochondrial branched-chain aminotransferase.", "journal": "Acta Crystallogr D Biol Crystallogr", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11264579/" }, { "id": "PMID:12269802", "title": "Crystal structures of human mitochondrial branched chain aminotransferase reaction intermediates: ketimine and pyridoxamine phosphate forms", "journal": "Biochemistry", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12269802/" } ], "x": 6800, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-508189", "label": "a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate leu, ile, or val + alpha-ketoglutarate [BCAT1]", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18323", "RHEA:24803", "RHEA:24815" ], "source_url": "https://reactome.org/content/detail/R-HSA-508189", "summary": "a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate leu, ile, or val + alpha-ketoglutarate [BCAT1] is a Reactome release 97 curated reaction event. The source record connects KIC,KMVA,KIV, L-Glu to Leu, Ile, Val, 2OG in cytosol and cross-references RHEA:18323, RHEA:24803, RHEA:24815.", "equation": "KIC,KMVA,KIV + L-Glu → Leu, Ile, Val + 2OG", "inputs": [ { "node_id": "R-ALL-508187", "reactome_id": "R-ALL-508187", "label": "KIC,KMVA,KIV", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-508182", "reactome_id": "R-ALL-508182", "label": "Leu, Ile, Val", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "branched-chain-amino-acid:2-oxoglutarate transaminase activity of BCAT1 dimer [cytosol]", "activity_id": "70700", "ec_numbers": [ "2.6.1.42" ], "accessions": null } ], "citations": [ { "id": "PMID:16141215", "title": "Structural determinants for branched-chain aminotransferase isozyme-specific inhibition by the anticonvulsant drug gabapentin", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16141215/" } ], "x": 6521.333333333333, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-508308", "label": "beta-methylglutaconyl-CoA + ADP + orthophosphate beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA)", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:13591" ], "source_url": "https://reactome.org/content/detail/R-HSA-508308", "summary": "beta-methylglutaconyl-CoA + ADP + orthophosphate beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA) is a Reactome release 97 curated reaction event. The source record connects bMG-CoA, ADP, Pi to bMC-CoA, ATP, HCO3- in mitochondrial matrix and cross-references RHEA:13591.", "equation": "bMG-CoA + ADP + Pi + H+ → bMC-CoA + ATP + HCO3-", "inputs": [ { "node_id": "R-ALL-70772", "reactome_id": "R-ALL-70772", "label": "bMG-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57346", "formula": "C27H37N7O19P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-70744", "reactome_id": "R-ALL-70744", "label": "bMC-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57344", "formula": "C26H38N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29896", "reactome_id": "R-ALL-29896", "label": "HCO3-", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "methylcrotonoyl-CoA carboxylase activity of 6x(Btn-MCCC1:MCCC2) [mitochondrial matrix]", "activity_id": "70771", "ec_numbers": [ "6.4.1.4" ], "accessions": null } ], "citations": [ { "id": "PMID:11181649", "title": "The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency.", "journal": "J Clin Invest", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11181649/" }, { "id": "PMID:11406611", "title": "Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency.", "journal": "Hum Mol Genet", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11406611/" } ], "x": 6521.333333333333, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-508369", "label": "alpha-methylacetoacetyl-CoA + NADH + H+ alpha-methyl-beta-hydroxybutyryl-CoA + NAD+", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:13283" ], "source_url": "https://reactome.org/content/detail/R-HSA-508369", "summary": "alpha-methylacetoacetyl-CoA + NADH + H+ alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ is a Reactome release 97 curated reaction event. The source record connects 2MACA-CoA, NADH, H+ to aMbHBUT-CoA, NAD+ in mitochondrial matrix and cross-references RHEA:13283.", "equation": "2MACA-CoA + NADH + H+ → aMbHBUT-CoA + NAD+", "inputs": [ { "node_id": "R-ALL-70836", "reactome_id": "R-ALL-70836", "label": "2MACA-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57335", "formula": "C26H38N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-70829", "reactome_id": "R-ALL-70829", "label": "aMbHBUT-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57312", "formula": "C26H40N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of HSD17B10 tetramer [mitochondrial matrix]", "activity_id": "70835", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:15342248", "title": "Crystal structure of human ABAD/HSD10 with a bound inhibitor: implications for design of Alzheimer's disease therapeutics", "journal": "J Mol Biol", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15342248/" }, { "id": "PMID:12696021", "title": "2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene.", "journal": "Am J Hum Genet", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12696021/" } ], "x": 7078.666666666667, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-508473", "label": "methylmalonyl semialdehyde + NADH + H+ beta-hydroxyisobutyrate + NAD+", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31277" ], "source_url": "https://reactome.org/content/detail/R-HSA-508473", "summary": "methylmalonyl semialdehyde + NADH + H+ beta-hydroxyisobutyrate + NAD+ is a Reactome release 97 curated reaction event. The source record connects 2M3OPROA, NADH, H+ to NAD+, bHIBA in mitochondrial matrix and cross-references RHEA:31277.", "equation": "2M3OPROA + NADH + H+ → NAD+ + bHIBA", "inputs": [ { "node_id": "R-ALL-30004", "reactome_id": "R-ALL-30004", "label": "2M3OPROA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62413", "formula": "C4H5O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-573404", "reactome_id": "R-ALL-573404", "label": "bHIBA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62638", "formula": "C4H7O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "3-hydroxyisobutyrate dehydrogenase activity of HIBADH tetramer [mitochondrial matrix]", "activity_id": "70884", "ec_numbers": [ "1.1.1.31" ], "accessions": null } ], "citations": [ { "id": "PMID:13416257", "title": "Purification and properties of beta-hydroxyisobutyric dehydrogenase", "journal": "J Biol Chem", "year": 1957, "url": "https://pubmed.ncbi.nlm.nih.gov/13416257/" } ], "x": 6521.333333333333, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-514604", "label": "GMP + NADPH + 2 H+ =\u003e IMP + NADP+ + NH4+ (GMPR,GMPR2)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17187" ], "source_url": "https://reactome.org/content/detail/R-HSA-514604", "summary": "GMP + NADPH + 2 H+ =\u003e IMP + NADP+ + NH4+ (GMPR,GMPR2) is a Reactome release 97 curated reaction event. The source record connects GMP, NADPH, H+ to NADP+, NH4+, IMP in cytosol and cross-references RHEA:17187.", "equation": "GMP + NADPH + 2 H+ → NADP+ + NH4+ + IMP", "inputs": [ { "node_id": "R-ALL-113578", "reactome_id": "R-ALL-113578", "label": "GMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58115", "formula": "C10H12N5O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29602", "reactome_id": "R-ALL-29602", "label": "IMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58053", "formula": "C10H11N4O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "GMP reductase activity of GMPR tetramers [cytosol]", "activity_id": "514632", "ec_numbers": [ "1.7.1.7" ], "accessions": null } ], "citations": [ { "id": "PMID:12009299", "title": "NADPH-dependent GMP reductase isoenzyme of human (GMPR2). Expression, purification, and kinetic properties", "journal": "Int J Biochem Cell Biol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12009299/" }, { "id": "PMID:16359702", "title": "Crystal structure of human guanosine monophosphate reductase 2 (GMPR2) in complex with GMP", "journal": "J Mol Biol", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16359702/" }, { "id": "PMID:218932", "title": "Reaction mechanism and specificity of human GMP reductase. Substrates, inhibitors, activators, and inactivators", "journal": "J Biol Chem", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/218932/" } ], "x": 3321.3333333333335, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-517444", "label": "TAT aminates HPP", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15095" ], "source_url": "https://reactome.org/content/detail/R-HSA-517444", "summary": "TAT aminates HPP is a Reactome release 97 curated reaction event. The source record connects HPP, L-Glu to L-Tyr, 2OG in cytosol and cross-references RHEA:15095.", "equation": "HPP + L-Glu → L-Tyr + 2OG", "inputs": [ { "node_id": "R-ALL-31365", "reactome_id": "R-ALL-31365", "label": "HPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36242", "formula": "C9H7O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29506", "reactome_id": "R-ALL-29506", "label": "L-Tyr", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58315", "formula": "C9H11NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-tyrosine:2-oxoglutarate transaminase activity of TAT dimer [cytosol]", "activity_id": "71154", "ec_numbers": [ "2.6.1.5" ], "accessions": null } ], "citations": [ { "id": "PMID:21153519", "title": "Tyrosine aminotransferase: biochemical and structural properties and molecular dynamics simulations", "journal": "Protein Cell", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/21153519/" }, { "id": "PMID:16640556", "title": "The narrow substrate specificity of human tyrosine aminotransferase--the enzyme deficient in tyrosinemia type II", "journal": "FEBS J", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16640556/" }, { "id": "PMID:16602095", "title": "The genetic tyrosinemias", "journal": "Am J Med Genet C Semin Med Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16602095/" } ], "x": 6800, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-548843", "label": "ACSL3,4 ligate CoA to AA to form AA-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:19714" ], "source_url": "https://reactome.org/content/detail/R-HSA-548843", "summary": "ACSL3,4 ligate CoA to AA to form AA-CoA is a Reactome release 97 curated reaction event. The source record connects AA, ATP, CoA-SH to AA-CoA, AMP, PPi in cytosol · endoplasmic reticulum membrane and cross-references RHEA:19714.", "equation": "AA + ATP + CoA-SH → AA-CoA + AMP + PPi + H2O", "inputs": [ { "node_id": "R-ALL-29768", "reactome_id": "R-ALL-29768", "label": "AA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:32395", "formula": "C20H31O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-548819", "reactome_id": "R-ALL-548819", "label": "AA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57368", "formula": "C41H62N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "long-chain fatty acid-CoA ligase activity of ACSL3,4 [endoplasmic reticulum membrane]", "activity_id": "548838", "ec_numbers": [ "6.2.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:12525535", "title": "A third MRX family (MRX68) is the result of mutation in the long chain fatty acid-CoA ligase 4 (FACL4) gene: proposal of a rapid enzymatic assay for screening mentally retarded patients", "journal": "J Med Genet", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12525535/" }, { "id": "PMID:11889465", "title": "FACL4, encoding fatty acid-CoA ligase 4, is mutated in nonspecific X-linked mental retardation", "journal": "Nat Genet", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11889465/" }, { "id": "PMID:18003621", "title": "Long chain acyl-CoA synthetase 3-mediated phosphatidylcholine synthesis is required for assembly of very low density lipoproteins in human hepatoma Huh7 cells", "journal": "J. Biol. Chem.", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18003621/" } ], "x": 1251.3333333333333, "y": 1857.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5652172", "label": "AKR1B1 reduces Glc to D-sorbitol", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "fructose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:59926" ], "source_url": "https://reactome.org/content/detail/R-HSA-5652172", "summary": "AKR1B1 reduces Glc to D-sorbitol is a Reactome release 97 curated reaction event. The source record connects Glc, NADPH, H+ to D-sorbitol, NADP+ in cytosol and cross-references RHEA:59926.", "equation": "Glc + NADPH + H+ → D-sorbitol + NADP+", "inputs": [ { "node_id": "R-ALL-70113", "reactome_id": "R-ALL-70113", "label": "Glc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:4167", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-5652213", "reactome_id": "R-ALL-5652213", "label": "D-sorbitol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17924", "formula": "C6H14O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aldose reductase (NADPH) activity of AKR1B1 [cytosol]", "activity_id": "196106", "ec_numbers": [ "1.1.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:1420136", "title": "Aldose reductase: model for a new paradigm of enzymic perfection in detoxification catalysts", "journal": "Biochemistry", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1420136/" }, { "id": "PMID:14401390", "title": "[Aldose reductase]", "journal": "Biochim. Biophys. Acta", "year": 1960, "url": "https://pubmed.ncbi.nlm.nih.gov/14401390/" }, { "id": "PMID:8281941", "title": "Sequence of pig lens aldose reductase and electrospray mass spectrometry of non-covalent and covalent complexes", "journal": "Eur. J. Biochem.", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8281941/" }, { "id": "PMID:2112546", "title": "Cloning and expression of human aldose reductase", "journal": "J. Biol. Chem.", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2112546/" }, { "id": "PMID:18220710", "title": "Aldose reductase, still a compelling target for diabetic neuropathy", "journal": "Curr Drug Targets", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18220710/" }, { "id": "PMID:15272156", "title": "The crystallographic structure of the aldose reductase-IDD552 complex shows direct proton donation from tyrosine 48", "journal": "Acta Crystallogr. D Biol. Crystallogr.", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15272156/" } ], "x": 2660, "y": 327.8333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5652195", "label": "SORD oxidizes D-sorbitol to Fru", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "fructose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:33033" ], "source_url": "https://reactome.org/content/detail/R-HSA-5652195", "summary": "SORD oxidizes D-sorbitol to Fru is a Reactome release 97 curated reaction event. The source record connects D-sorbitol, NAD+ to NADH, H+, keto-D-Fru in cytosol and cross-references RHEA:33033.", "equation": "D-sorbitol + NAD+ → NADH + H+ + keto-D-Fru", "inputs": [ { "node_id": "R-ALL-5652213", "reactome_id": "R-ALL-5652213", "label": "D-sorbitol", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17924", "formula": "C6H14O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9832975", "reactome_id": "R-ALL-9832975", "label": "keto-D-Fru", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:48095", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-iditol 2-dehydrogenase (NAD+) activity of SORD tetramer [cytosol]", "activity_id": "5652209", "ec_numbers": [ "1.1.1.14" ], "accessions": null } ], "citations": [ { "id": "PMID:14401391", "title": "[The mechanism of the formation of seminal fructose and fetal fructose]", "journal": "Biochim. Biophys. Acta", "year": 1960, "url": "https://pubmed.ncbi.nlm.nih.gov/14401391/" }, { "id": "PMID:2691249", "title": "Variability within mammalian sorbitol dehydrogenases. The primary structure of the human liver enzyme", "journal": "Eur. J. Biochem.", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2691249/" }, { "id": "PMID:6870831", "title": "Polyol-pathway enzymes of human brain. Partial purification and properties of sorbitol dehydrogenase", "journal": "Biochem. J.", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/6870831/" }, { "id": "PMID:12962626", "title": "X-ray crystallographic and kinetic studies of human sorbitol dehydrogenase", "journal": "Structure", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12962626/" } ], "x": 2938.666666666667, "y": 327.8333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5690565", "label": "SCD desaturates ST-CoA to OLE-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:19722" ], "source_url": "https://reactome.org/content/detail/R-HSA-5690565", "summary": "SCD desaturates ST-CoA to OLE-CoA is a Reactome release 97 curated reaction event. The source record connects ST-CoA, H+, O2 to OLE-CoA, Fe(3+)Cb5, H2O in endoplasmic reticulum membrane · cytosol and cross-references RHEA:19722.", "equation": "ST-CoA + 2 H+ + O2 + 2 Fe(2+)Cb5 → OLE-CoA + 2 Fe(3+)Cb5 + 2 H2O", "inputs": [ { "node_id": "R-ALL-428139", "reactome_id": "R-ALL-428139", "label": "ST-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57394", "formula": "C39H66N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-4085415", "reactome_id": "R-ALL-4085415", "label": "Fe(2+)Cb5", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:16518", "formula": "", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-5690558", "reactome_id": "R-ALL-5690558", "label": "OLE-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57387", "formula": "C39H64N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-4085425", "reactome_id": "R-ALL-4085425", "label": "Fe(3+)Cb5", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:18097", "formula": "", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "stearoyl-CoA 9-desaturase activity of SCD dimer [endoplasmic reticulum membrane]", "activity_id": "5690564", "ec_numbers": [ "1.14.19.1" ], "accessions": null } ], "citations": [ { "id": "PMID:7909540", "title": "Partial characterization of a cDNA for human stearoyl-CoA desaturase and changes in its mRNA expression in some normal and malignant tissues", "journal": "Int J Cancer", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7909540/" }, { "id": "PMID:10229681", "title": "Human stearoyl-CoA desaturase: alternative transcripts generated from a single gene by usage of tandem polyadenylation sites", "journal": "Biochem. J.", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10229681/" }, { "id": "PMID:15610069", "title": "Characterization of human SCD2, an oligomeric desaturase with improved stability and enzyme activity by cross-linking in intact cells", "journal": "Biochem. J.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15610069/" } ], "x": 1530, "y": 1857.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5694137", "label": "PXLP-K280-SHMT2 tetramer transfers CH2OH group to Gly to form L-Ser", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:15482" ], "source_url": "https://reactome.org/content/detail/R-HSA-5694137", "summary": "PXLP-K280-SHMT2 tetramer transfers CH2OH group to Gly to form L-Ser is a Reactome release 97 curated reaction event. The source record connects 5,10-methylene-THF, Gly, H2O to THF, L-Ser in mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:15482.", "equation": "5,10-methylene-THF + Gly + H2O → THF + L-Ser", "inputs": [ { "node_id": "R-ALL-5693984", "reactome_id": "R-ALL-5693984", "label": "5,10-methylene-THF", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15636", "formula": "C20H21N7O6", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159549", "reactome_id": "R-ALL-159549", "label": "Gly", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-200671", "reactome_id": "R-ALL-200671", "label": "THF", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57453", "formula": "C19H21N7O6", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-379732", "reactome_id": "R-ALL-379732", "label": "L-Ser", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33384", "formula": "C3H7NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glycine hydroxymethyltransferase activity of PXLP-K280-SHMT2 tetramer [mitochondrial inner membrane]", "activity_id": "5694152", "ec_numbers": [ "2.1.2.1" ], "accessions": null } ], "citations": [ { "id": "PMID:21876188", "title": "Identification of a de novo thymidylate biosynthesis pathway in mammalian mitochondria", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21876188/" } ], "x": 5008.666666666667, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5694494", "label": "AACS ligates CoA-SH to ACA, forming ACA-CoA", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:16118" ], "source_url": "https://reactome.org/content/detail/R-HSA-5694494", "summary": "AACS ligates CoA-SH to ACA, forming ACA-CoA is a Reactome release 97 curated reaction event. The source record connects ACA, ATP, CoA-SH to ACA-CoA, AMP, PPi in cytosol and cross-references RHEA:16118.", "equation": "ACA + ATP + CoA-SH → ACA-CoA + AMP + PPi", "inputs": [ { "node_id": "R-ALL-29666", "reactome_id": "R-ALL-29666", "label": "ACA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:13705", "formula": "C4H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-191293", "reactome_id": "R-ALL-191293", "label": "ACA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57286", "formula": "C25H36N7O18P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acetoacetate-CoA ligase activity of AACS [cytosol]", "activity_id": "5694501", "ec_numbers": [ "6.2.1.16" ], "accessions": null } ], "citations": [ { "id": "PMID:20102333", "title": "Transcriptional regulation of the human acetoacetyl-CoA synthetase gene by PPARgamma", "journal": "Biochem. J.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20102333/" }, { "id": "PMID:12623130", "title": "Expression of acetoacetyl-CoA synthetase, a novel cytosolic ketone body-utilizing enzyme, in human brain", "journal": "Biochem. Pharmacol.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12623130/" }, { "id": "PMID:7061490", "title": "Lipogenesis from ketone bodies in the isolated perfused rat liver. Evidence for the cytosolic activation of acetoacetate", "journal": "J. Biol. Chem.", "year": 1982, "url": "https://pubmed.ncbi.nlm.nih.gov/7061490/" }, { "id": "PMID:23396451", "title": "Ketone body metabolism and cardiovascular disease", "journal": "Am. J. Physiol. Heart Circ. Physiol.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23396451/" } ], "x": 7461.333333333333, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5695957", "label": "ACSBG1,2 ligates CoA-SH to VLCFA, forming VLCFA-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:54537" ], "source_url": "https://reactome.org/content/detail/R-HSA-5695957", "summary": "ACSBG1,2 ligates CoA-SH to VLCFA, forming VLCFA-CoA is a Reactome release 97 curated reaction event. The source record connects VLCFA, ATP, CoA-SH to VLCFA-CoA, AMP, PPi in cytosol and cross-references RHEA:54537.", "equation": "VLCFA + ATP + CoA-SH → VLCFA-CoA + AMP + PPi", "inputs": [ { "node_id": "R-ALL-5695985", "reactome_id": "R-ALL-5695985", "label": "VLCFA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58950", "formula": "CO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-5695971", "reactome_id": "R-ALL-5695971", "label": "VLCFA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:138261", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "very long-chain fatty acid-CoA ligase activity of ACSBG1,2 [cytosol]", "activity_id": "5695982", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10954726", "title": "Very long-chain acyl-CoA synthetases. Human \"bubblegum\" represents a new family of proteins capable of activating very long-chain fatty acids", "journal": "J. Biol. Chem.", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10954726/" }, { "id": "PMID:12975357", "title": "The acyl-CoA synthetase \"bubblegum\" (lipidosin): further characterization and role in neuronal fatty acid beta-oxidation.", "journal": "J. Biol. Chem.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12975357/" }, { "id": "PMID:16371355", "title": "The second member of the human and murine bubblegum family is a testis- and brainstem-specific acyl-CoA synthetase", "journal": "J. Biol. Chem.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16371355/" } ], "x": 1251.3333333333333, "y": 1597.8333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5696007", "label": "ACSF3 ligates CoA-SH to VLCFA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:54537" ], "source_url": "https://reactome.org/content/detail/R-HSA-5696007", "summary": "ACSF3 ligates CoA-SH to VLCFA is a Reactome release 97 curated reaction event. The source record connects VLCFA, ATP, CoA-SH to VLCFA-CoA, AMP, PPi in mitochondrial matrix and cross-references RHEA:54537.", "equation": "VLCFA + ATP + CoA-SH → VLCFA-CoA + AMP + PPi", "inputs": [ { "node_id": "R-ALL-5696008", "reactome_id": "R-ALL-5696008", "label": "VLCFA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58950", "formula": "CO2R", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-5696003", "reactome_id": "R-ALL-5696003", "label": "VLCFA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:138261", "formula": "C22H31N7O17P3SR", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159448", "reactome_id": "R-ALL-159448", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "very long-chain fatty acid-CoA ligase activity of ACSF3 [mitochondrial matrix]", "activity_id": "5696000", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:17762044", "title": "Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome", "journal": "J. Lipid Res.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17762044/" } ], "x": 1530, "y": 1597.8333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5696080", "label": "ALDH3B1 oxidises HXAL to PALM", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "plasma membrane · cytosol", "compartment_ids": [ "GO:0005886", "GO:0005829" ], "rhea_ids": [ "RHEA:33741" ], "source_url": "https://reactome.org/content/detail/R-HSA-5696080", "summary": "ALDH3B1 oxidises HXAL to PALM is a Reactome release 97 curated reaction event. The source record connects HXAL, H2O, NAD+ to PALM, NADH, H+ in plasma membrane · cytosol and cross-references RHEA:33741.", "equation": "HXAL + H2O + NAD+ → PALM + NADH + H+", "inputs": [ { "node_id": "R-ALL-428663", "reactome_id": "R-ALL-428663", "label": "HXAL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17600", "formula": "C16H32O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-3632881", "reactome_id": "R-ALL-3632881", "label": "PALM", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:7896", "formula": "C16H31O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aldehyde dehydrogenase [NAD(P)+] activity of ALDH3B1 [plasma membrane]", "activity_id": "5696087", "ec_numbers": [ "1.2.1.5" ], "accessions": null } ], "citations": [ { "id": "PMID:23721920", "title": "Substrate specificity, plasma membrane localization, and lipid modification of the aldehyde dehydrogenase ALDH3B1", "journal": "Biochim. Biophys. Acta", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23721920/" }, { "id": "PMID:20699116", "title": "Molecular characterization, expression analysis, and role of ALDH3B1 in the cellular protection against oxidative stress", "journal": "Free Radic. Biol. Med.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20699116/" }, { "id": "PMID:17382292", "title": "Expression and initial characterization of human ALDH3B1", "journal": "Biochem Biophys Res Commun", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17382292/" } ], "x": 3321.3333333333335, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5696415", "label": "PXLP-K278-ETNPPL tetramer hydrolyses PETA", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:17890" ], "source_url": "https://reactome.org/content/detail/R-HSA-5696415", "summary": "PXLP-K278-ETNPPL tetramer hydrolyses PETA is a Reactome release 97 curated reaction event. The source record connects PETA, H2O to CH3CHO, NH3, Pi in mitochondrial matrix and cross-references RHEA:17890.", "equation": "PETA + H2O → CH3CHO + NH3 + Pi", "inputs": [ { "node_id": "R-ALL-5696414", "reactome_id": "R-ALL-5696414", "label": "PETA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17553", "formula": "C2H8NO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113532", "reactome_id": "R-ALL-113532", "label": "CH3CHO", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15343", "formula": "C2H4O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-5693978", "reactome_id": "R-ALL-5693978", "label": "NH3", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16134", "formula": "H3N", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "ethanolamine-phosphate phospho-lyase activity of PXLP-K278-ETNPPL tetramer [mitochondrial matrix]", "activity_id": "5696410", "ec_numbers": [ "4.2.3.2" ], "accessions": null } ], "citations": [ { "id": "PMID:22241472", "title": "Molecular identification of hydroxylysine kinase and of ammoniophospholyases acting on 5-phosphohydroxy-L-lysine and phosphoethanolamine", "journal": "J. Biol. Chem.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22241472/" } ], "x": 2381.3333333333335, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-5696457", "label": "BDH2 dehydrogenates 3HBA", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:20522" ], "source_url": "https://reactome.org/content/detail/R-HSA-5696457", "summary": "BDH2 dehydrogenates 3HBA is a Reactome release 97 curated reaction event. The source record connects 3HBA, NAD+ to ACA, H+, NADH in cytosol and cross-references RHEA:20522.", "equation": "3HBA + NAD+ → ACA + H+ + NADH", "inputs": [ { "node_id": "R-ALL-5696461", "reactome_id": "R-ALL-5696461", "label": "3HBA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:10983", "formula": "C4H7O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29666", "reactome_id": "R-ALL-29666", "label": "ACA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13705", "formula": "C4H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "3-hydroxybutyrate dehydrogenase activity of BDH2 tetramer [cytosol]", "activity_id": "5696453", "ec_numbers": [ "1.1.1.30" ], "accessions": null } ], "citations": [ { "id": "PMID:16380372", "title": "Characterization of human DHRS6, an orphan short chain dehydrogenase/reductase enzyme: a novel, cytosolic type 2 R-beta-hydroxybutyrate dehydrogenase", "journal": "J. Biol. Chem.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16380372/" } ], "x": 8018.666666666667, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6783939", "label": "PYCR2 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14111" ], "source_url": "https://reactome.org/content/detail/R-HSA-6783939", "summary": "PYCR2 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro is a Reactome release 97 curated reaction event. The source record connects 1PYR-5COOH, H+, NADPH to L-Pro, NADP+ in cytosol and cross-references RHEA:14111.", "equation": "1PYR-5COOH + 2 H+ + NADPH → L-Pro + NADP+", "inputs": [ { "node_id": "R-ALL-35305", "reactome_id": "R-ALL-35305", "label": "1PYR-5COOH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17388", "formula": "C5H6NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113538", "reactome_id": "R-ALL-113538", "label": "L-Pro", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:60039", "formula": "C5H9NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "pyrroline-5-carboxylate reductase activity of PYCR2 decamer [cytosol]", "activity_id": "6783944", "ec_numbers": [ "1.5.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:23024808", "title": "Functional specialization in proline biosynthesis of melanoma", "journal": "PLoS ONE", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/23024808/" }, { "id": "PMID:9514741", "title": "Crystal structure of human recombinant ornithine aminotransferase.", "journal": "J Mol Biol", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9514741/" }, { "id": "PMID:2722838", "title": "Purified human erythrocyte pyrroline-5-carboxylate reductase. Preferential oxidation of NADPH.", "journal": "J Biol Chem", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2722838/" }, { "id": "PMID:25865492", "title": "Mutations in PYCR2, Encoding Pyrroline-5-Carboxylate Reductase 2, Cause Microcephaly and Hypomyelination", "journal": "Am. J. Hum. Genet.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25865492/" } ], "x": 7461.333333333333, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6783955", "label": "PYCR3 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14111" ], "source_url": "https://reactome.org/content/detail/R-HSA-6783955", "summary": "PYCR3 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro is a Reactome release 97 curated reaction event. The source record connects 1PYR-5COOH, H+, NADPH to L-Pro, NADP+ in cytosol and cross-references RHEA:14111.", "equation": "1PYR-5COOH + 2 H+ + NADPH → L-Pro + NADP+", "inputs": [ { "node_id": "R-ALL-35305", "reactome_id": "R-ALL-35305", "label": "1PYR-5COOH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17388", "formula": "C5H6NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113538", "reactome_id": "R-ALL-113538", "label": "L-Pro", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:60039", "formula": "C5H9NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "pyrroline-5-carboxylate reductase activity of PYCR3 decamer [cytosol]", "activity_id": "6783951", "ec_numbers": [ "1.5.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:23024808", "title": "Functional specialization in proline biosynthesis of melanoma", "journal": "PLoS ONE", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/23024808/" }, { "id": "PMID:2722838", "title": "Purified human erythrocyte pyrroline-5-carboxylate reductase. Preferential oxidation of NADPH.", "journal": "J Biol Chem", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2722838/" } ], "x": 7740, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6784224", "label": "PRODH2:FAD dimer dehydrogenates HPRO to 1-pyrroline-3-hydroxy-5-carboxylate", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:52513" ], "source_url": "https://reactome.org/content/detail/R-HSA-6784224", "summary": "PRODH2:FAD dimer dehydrogenates HPRO to 1-pyrroline-3-hydroxy-5-carboxylate is a Reactome release 97 curated reaction event. The source record connects HPRO, quinone to 1PYR-3OH-5COOH, quinol, H+ in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:52513.", "equation": "HPRO + quinone → 1PYR-3OH-5COOH + quinol + H+", "inputs": [ { "node_id": "R-ALL-6784215", "reactome_id": "R-ALL-6784215", "label": "HPRO", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58375", "formula": "C5H9NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9930628", "reactome_id": "R-ALL-9930628", "label": "quinone", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:132124", "formula": "C6O2R4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9929425", "reactome_id": "R-ALL-9929425", "label": "1PYR-3OH-5COOH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62612", "formula": "C5H6NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9930630", "reactome_id": "R-ALL-9930630", "label": "quinol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:24646", "formula": "C6H2O2R4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "proline dehydrogenase activity of PRODH2 dimer [mitochondrial inner membrane]", "activity_id": "6784234", "ec_numbers": [ "1.5.5.2" ], "accessions": null } ], "citations": [ { "id": "PMID:13681368", "title": "Hydroxyproline metabolism. II. Enzymatic preparation and properties of Delta 1-pyrroline-3-hydroxy-5-carboxylic acid", "journal": "J. Biol. Chem.", "year": 1960, "url": "https://pubmed.ncbi.nlm.nih.gov/13681368/" }, { "id": "PMID:6250440", "title": "Metabolism of proline and the hydroxyprolines", "journal": "Annu Rev Biochem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6250440/" }, { "id": "PMID:12865426", "title": "Proteomic analysis of the mouse liver mitochondrial inner membrane", "journal": "J. Biol. Chem.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12865426/" }, { "id": "PMID:25697095", "title": "Proline dehydrogenase 2 (PRODH2) is a hydroxyproline dehydrogenase (HYPDH) and molecular target for treating primary hyperoxaluria", "journal": "Biochem. J.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25697095/" } ], "x": 868.6666666666667, "y": 3237, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6784402", "label": "ALDH4A1 converts 1PYR-3OH-5COOH to 4-OH-L-glutamate", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31156" ], "source_url": "https://reactome.org/content/detail/R-HSA-6784402", "summary": "ALDH4A1 converts 1PYR-3OH-5COOH to 4-OH-L-glutamate is a Reactome release 97 curated reaction event. The source record connects H2O, 1PYR-3OH-5COOH, NAD+ to 4OH-L-Glu, H+, NADH in mitochondrial matrix and cross-references RHEA:31156.", "equation": "2 H2O + 1PYR-3OH-5COOH + NAD+ → 4OH-L-Glu + H+ + NADH", "inputs": [ { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9929425", "reactome_id": "R-ALL-9929425", "label": "1PYR-3OH-5COOH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62612", "formula": "C5H6NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-9929459", "reactome_id": "R-ALL-9929459", "label": "4OH-L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:6331", "formula": "C5H8NO5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-glutamate gamma-semialdehyde dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]", "activity_id": "70678", "ec_numbers": [ "1.2.1.88" ], "accessions": null } ], "citations": [ { "id": "PMID:13681370", "title": "Hydroxyproline metabolism. IV. Enzymatic synthesis of gamma-hydroxyglutamate from Delta 1-pyrroline-3-hydroxy-5-carboxylate", "journal": "J Biol Chem", "year": 1960, "url": "https://pubmed.ncbi.nlm.nih.gov/13681370/" }, { "id": "PMID:22516612", "title": "The three-dimensional structural basis of type II hyperprolinemia", "journal": "J. Mol. Biol.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22516612/" }, { "id": "PMID:500817", "title": "Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline", "journal": "J Clin Invest", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/500817/" } ], "x": 590, "y": 3160.5555555555557, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6785928", "label": "CHAC1,2 cleaves GSH to OPRO and CysGly", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:47725" ], "source_url": "https://reactome.org/content/detail/R-HSA-6785928", "summary": "CHAC1,2 cleaves GSH to OPRO and CysGly is a Reactome release 97 curated reaction event. The source record connects GSH to OPRO, CysGly in cytosol and cross-references RHEA:47725.", "equation": "GSH → OPRO + CysGly", "inputs": [ { "node_id": "R-ALL-29450", "reactome_id": "R-ALL-29450", "label": "GSH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57925", "formula": "C10H16N3O6S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1247942", "reactome_id": "R-ALL-1247942", "label": "OPRO", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58402", "formula": "C5H6NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1247904", "reactome_id": "R-ALL-1247904", "label": "CysGly", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:61694", "formula": "C5H10N2O3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glutathione specific gamma-glutamylcyclotransferase activity of CHAC1,2 [cytosol]", "activity_id": "6785934", "ec_numbers": [ "4.3.2.7" ], "accessions": null } ], "citations": [ { "id": "PMID:19109178", "title": "CHAC1/MGC4504 is a novel proapoptotic component of the unfolded protein response, downstream of the ATF4-ATF3-CHOP cascade", "journal": "J. Immunol.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19109178/" }, { "id": "PMID:25931127", "title": "Human CHAC1 Protein Degrades Glutathione, and mRNA Induction Is Regulated by the Transcription Factors ATF4 and ATF3 and a Bipartite ATF/CRE Regulatory Element", "journal": "J. Biol. Chem.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25931127/" } ], "x": 868.6666666666667, "y": 3556.333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6785933", "label": "UGT8 transfers Gal from UDP-Gal to CERA", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:48344" ], "source_url": "https://reactome.org/content/detail/R-HSA-6785933", "summary": "UGT8 transfers Gal from UDP-Gal to CERA is a Reactome release 97 curated reaction event. The source record connects CERA, UDP-Gal to GalCer, UDP, H+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:48344.", "equation": "CERA + UDP-Gal → GalCer + UDP + H+", "inputs": [ { "node_id": "R-ALL-428256", "reactome_id": "R-ALL-428256", "label": "CERA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:83273", "formula": "C4H7NO3R2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1981137", "reactome_id": "R-ALL-1981137", "label": "UDP-Gal", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:66914", "formula": "C15H22N2O17P2", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-6785915", "reactome_id": "R-ALL-6785915", "label": "GalCer", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:36498", "formula": "C10H17NO8R2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-158602", "reactome_id": "R-ALL-158602", "label": "UDP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-156540", "reactome_id": "R-ALL-156540", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "N-acylsphingosine galactosyltransferase activity of UGT8 [endoplasmic reticulum membrane]", "activity_id": "6785918", "ec_numbers": [ "2.4.1.47" ], "accessions": null } ], "citations": [ { "id": "PMID:9125199", "title": "Cloning, characterization, and expression of human ceramide galactosyltransferase cDNA", "journal": "Biochem. Biophys. Res. Commun.", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9125199/" }, { "id": "PMID:23290777", "title": "Ceramide glycosylation catalyzed by glucosylceramide synthase and cancer drug resistance", "journal": "Adv. Cancer Res.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23290777/" }, { "id": "PMID:9748263", "title": "UDP-galactose:ceramide galactosyltransferase is a class I integral membrane protein of the endoplasmic reticulum", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9748263/" } ], "x": 3600, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6787321", "label": "DERA cleaves dR5P to GA3P and CH3CHO", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:12822" ], "source_url": "https://reactome.org/content/detail/R-HSA-6787321", "summary": "DERA cleaves dR5P to GA3P and CH3CHO is a Reactome release 97 curated reaction event. The source record connects dR5P to GA3P, CH3CHO in cytosol and cross-references RHEA:12822.", "equation": "dR5P → GA3P + CH3CHO", "inputs": [ { "node_id": "R-ALL-6787328", "reactome_id": "R-ALL-6787328", "label": "dR5P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62877", "formula": "C5H9O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29510", "reactome_id": "R-ALL-29510", "label": "CH3CHO", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15343", "formula": "C2H4O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "deoxyribose-phosphate aldolase activity of DERA [cytosol]", "activity_id": "6787320", "ec_numbers": [ "4.1.2.4" ], "accessions": null } ], "citations": [ { "id": "PMID:25229427", "title": "DERA is the human deoxyribose phosphate aldolase and is involved in stress response", "journal": "Biochim. Biophys. Acta", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/25229427/" } ], "x": 311.33333333333337, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6787329", "label": "PGM2:Mg2+ isomerises R1P to R5P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18794" ], "source_url": "https://reactome.org/content/detail/R-HSA-6787329", "summary": "PGM2:Mg2+ isomerises R1P to R5P is a Reactome release 97 curated reaction event. The source record connects R1P to R5P in cytosol and cross-references RHEA:18794.", "equation": "R1P → R5P", "inputs": [ { "node_id": "R-ALL-112016", "reactome_id": "R-ALL-112016", "label": "R1P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57720", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-73578", "reactome_id": "R-ALL-73578", "label": "R5P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:78346", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phosphopentomutase activity of PGM2:Mg2+ [cytosol]", "activity_id": "6787332", "ec_numbers": [ "5.4.2.7" ], "accessions": null } ], "citations": [ { "id": "PMID:17804405", "title": "Molecular identification of mammalian phosphopentomutase and glucose-1,6-bisphosphate synthase, two members of the alpha-D-phosphohexomutase family", "journal": "J Biol Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17804405/" } ], "x": 868.6666666666667, "y": 1082.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6788295", "label": "HDHD1:Mg2+ dephosphorylates PURIDP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10945" ], "source_url": "https://reactome.org/content/detail/R-HSA-6788295", "summary": "HDHD1:Mg2+ dephosphorylates PURIDP is a Reactome release 97 curated reaction event. The source record connects PURIDP, H2O to PURID, Pi in cytosol and cross-references RHEA:10945.", "equation": "PURIDP + H2O → PURID + Pi", "inputs": [ { "node_id": "R-ALL-6788291", "reactome_id": "R-ALL-6788291", "label": "PURIDP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58380", "formula": "C9H11N2O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6788290", "reactome_id": "R-ALL-6788290", "label": "PURID", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17802", "formula": "C9H12N2O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "hydrolase activity of HDHD1:Mg2+ [cytosol]", "activity_id": "6788289", "ec_numbers": [ "3" ], "accessions": null } ], "citations": [ { "id": "PMID:20722631", "title": "HDHD1, which is often deleted in X-linked ichthyosis, encodes a pseudouridine-5'-phosphatase", "journal": "Biochem. J.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20722631/" } ], "x": 3321.3333333333335, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6788597", "label": "HMGCLL1:Mg2+ cleaves bHMG-CoA to Ac-CoA and ACA", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24405" ], "source_url": "https://reactome.org/content/detail/R-HSA-6788597", "summary": "HMGCLL1:Mg2+ cleaves bHMG-CoA to Ac-CoA and ACA is a Reactome release 97 curated reaction event. The source record connects bHMG-CoA to Ac-CoA, ACA in cytosol and cross-references RHEA:24405.", "equation": "bHMG-CoA → Ac-CoA + ACA", "inputs": [ { "node_id": "R-ALL-191374", "reactome_id": "R-ALL-191374", "label": "bHMG-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43074", "formula": "C27H39N7O20P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29666", "reactome_id": "R-ALL-29666", "label": "ACA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13705", "formula": "C4H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "hydroxymethylglutaryl-CoA lyase activity of HMGCLL1:Mg2+ [cytosol]", "activity_id": "6788588", "ec_numbers": [ "4.1.3.4" ], "accessions": null } ], "citations": [ { "id": "PMID:22865860", "title": "Identification and characterization of an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase", "journal": "J. Biol. Chem.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22865860/" }, { "id": "PMID:22847177", "title": "Characterization of a novel HMG-CoA lyase enzyme with a dual location in endoplasmic reticulum and cytosol", "journal": "J. Lipid Res.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22847177/" } ], "x": 7740, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6788798", "label": "AK4 phosphorylates (d)NMPs to (d)NDPs", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:12974", "RHEA:23101", "RHEA:11601", "RHEA:25095" ], "source_url": "https://reactome.org/content/detail/R-HSA-6788798", "summary": "AK4 phosphorylates (d)NMPs to (d)NDPs is a Reactome release 97 curated reaction event. The source record connects (d)NMPs, ATP to (d)NDPs, ADP in mitochondrial matrix and cross-references RHEA:12974, RHEA:23101, RHEA:11601, RHEA:25095.", "equation": "(d)NMPs + ATP → (d)NDPs + ADP", "inputs": [ { "node_id": "R-ALL-6788802", "reactome_id": "R-ALL-6788802", "label": "(d)NMPs", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6788801", "reactome_id": "R-ALL-6788801", "label": "(d)NDPs", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside monophosphate kinase activity of AK4 [mitochondrial matrix]", "activity_id": "6788795", "ec_numbers": [ "2.7.4.4" ], "accessions": null } ], "citations": [ { "id": "PMID:19766732", "title": "Evidence of an intact N-terminal translocation sequence of human mitochondrial adenylate kinase 4", "journal": "Int. J. Biochem. Cell Biol.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/19766732/" } ], "x": 3878.666666666667, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6788810", "label": "AK6 phosphorylates (d)NMPs to (d)NDPs", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "nucleoplasm", "compartment_ids": [ "GO:0005654" ], "rhea_ids": [ "RHEA:23101", "RHEA:12974", "RHEA:11601", "RHEA:25095" ], "source_url": "https://reactome.org/content/detail/R-HSA-6788810", "summary": "AK6 phosphorylates (d)NMPs to (d)NDPs is a Reactome release 97 curated reaction event. The source record connects (d)NMPs, ATP to (d)NDPs, ADP in nucleoplasm and cross-references RHEA:23101, RHEA:12974, RHEA:11601, RHEA:25095.", "equation": "(d)NMPs + ATP → (d)NDPs + ADP", "inputs": [ { "node_id": "R-ALL-6788813", "reactome_id": "R-ALL-6788813", "label": "(d)NMPs", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "nucleoplasm", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29358", "reactome_id": "R-ALL-29358", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6788807", "reactome_id": "R-ALL-6788807", "label": "(d)NDPs", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "nucleoplasm", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113582", "reactome_id": "R-ALL-113582", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "nucleoplasm", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside monophosphate kinase activity of AK6 [nucleoplasm]", "activity_id": "6788811", "ec_numbers": [ "2.7.4.4" ], "accessions": null } ], "citations": [ { "id": "PMID:15630091", "title": "The crystal structure of human adenylate kinase 6: An adenylate kinase localized to the cell nucleus", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15630091/" } ], "x": 3878.666666666667, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6798317", "label": "GNMT tetramer transfers methyl group from AdoMet to Gly to form AdoHyc and SARC", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19938" ], "source_url": "https://reactome.org/content/detail/R-HSA-6798317", "summary": "GNMT tetramer transfers methyl group from AdoMet to Gly to form AdoHyc and SARC is a Reactome release 97 curated reaction event. The source record connects AdoMet, Gly to AdoHcy, SARC, H+ in cytosol and cross-references RHEA:19938.", "equation": "AdoMet + Gly → AdoHcy + SARC + H+", "inputs": [ { "node_id": "R-ALL-71284", "reactome_id": "R-ALL-71284", "label": "AdoMet", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59789", "formula": "C15H23N6O5S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29424", "reactome_id": "R-ALL-29424", "label": "Gly", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-71285", "reactome_id": "R-ALL-71285", "label": "AdoHcy", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57856", "formula": "C14H20N6O5S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-5359017", "reactome_id": "R-ALL-5359017", "label": "SARC", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57433", "formula": "C3H7NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycine N-methyltransferase activity of GNMT tetramer [cytosol]", "activity_id": "6798320", "ec_numbers": [ "2.1.1.20" ], "accessions": null } ], "citations": [ { "id": "PMID:15340920", "title": "Glycine N-methyltransferases: a comparison of the crystal structures and kinetic properties of recombinant human, mouse and rat enzymes", "journal": "Proteins", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15340920/" } ], "x": 311.33333333333337, "y": 3313.444444444445, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6798335", "label": "BPGM dimer isomerises 1,3BPG to 2,3BPG", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17766" ], "source_url": "https://reactome.org/content/detail/R-HSA-6798335", "summary": "BPGM dimer isomerises 1,3BPG to 2,3BPG is a Reactome release 97 curated reaction event. The source record connects 1,3BPG to 2,3BPG, H+ in cytosol and cross-references RHEA:17766.", "equation": "1,3BPG → 2,3BPG + H+", "inputs": [ { "node_id": "R-ALL-29800", "reactome_id": "R-ALL-29800", "label": "1,3BPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57604", "formula": "C3H4O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-6798337", "reactome_id": "R-ALL-6798337", "label": "2,3BPG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58248", "formula": "C3H3O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "bisphosphoglycerate mutase activity of BPGM dimer [cytosol]", "activity_id": "6798336", "ec_numbers": [ "5.4.2.4" ], "accessions": null } ], "citations": [ { "id": "PMID:5687724", "title": "The purification and properties of diphosphoglycerate mutase from human erythrocytes", "journal": "J. Biol. Chem.", "year": 1968, "url": "https://pubmed.ncbi.nlm.nih.gov/5687724/" }, { "id": "PMID:4555506", "title": "X-ray diffraction study of binding of 2,3-diphosphoglycerate to human deoxyhaemoglobin", "journal": "Nature", "year": 1972, "url": "https://pubmed.ncbi.nlm.nih.gov/4555506/" } ], "x": 868.6666666666667, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6799495", "label": "GLYCTK phosphorylates DGA to 3PDGA", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "fructose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23517" ], "source_url": "https://reactome.org/content/detail/R-HSA-6799495", "summary": "GLYCTK phosphorylates DGA to 3PDGA is a Reactome release 97 curated reaction event. The source record connects DGA, ATP to 3PDGA, ADP, H+ in cytosol and cross-references RHEA:23517.", "equation": "DGA + ATP → 3PDGA + ADP + H+", "inputs": [ { "node_id": "R-ALL-6799497", "reactome_id": "R-ALL-6799497", "label": "DGA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16659", "formula": "C3H5O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6799493", "reactome_id": "R-ALL-6799493", "label": "3PDGA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58272", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycerate kinase activity of GLYCTK [cytosol]", "activity_id": "6799496", "ec_numbers": [ "2.7.1.31" ], "accessions": null } ], "citations": [ { "id": "PMID:16753811", "title": "Isolation and characterization of the human D-glyceric acidemia related glycerate kinase gene GLYCTK1 and its alternatively splicing variant GLYCTK2", "journal": "DNA Seq.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16753811/" }, { "id": "PMID:2537226", "title": "D-glycerate kinase deficiency as a cause of D-glyceric aciduria", "journal": "FEBS Lett.", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2537226/" }, { "id": "PMID:20949620", "title": "D-glyceric aciduria is caused by genetic deficiency of D-glycerate kinase (GLYCTK)", "journal": "Hum. Mutat.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20949620/" } ], "x": 2381.3333333333335, "y": 457.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6799545", "label": "MAN2C1 hydrolyses GlcNAc (Man)9 to GlcNAc (Man)5", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "oligosaccharide-recovery" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-6799545", "summary": "MAN2C1 hydrolyses GlcNAc (Man)9 to GlcNAc (Man)5 is a Reactome release 97 curated reaction event. The source record connects alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-bet…, H2O to alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc, Man in cytosol.", "equation": "alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-bet… + 4 H2O → alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc + 4 Man", "inputs": [ { "node_id": "R-ALL-6799540", "reactome_id": "R-ALL-6799540", "label": "alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e2)-alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-bet…", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:91284", "formula": "C62H105NO51", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 4, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6799543", "reactome_id": "R-ALL-6799543", "label": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:91280", "formula": "C38H65NO31", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-429579", "reactome_id": "R-ALL-429579", "label": "Man", "role": "output", "stoichiometry": 4, "chebi_id": "CHEBI:4208", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "alpha-mannosidase activity of MAN2C1:Zn2+ [cytosol]", "activity_id": "6799539", "ec_numbers": [ "3.2.1.24" ], "accessions": null } ], "citations": [ { "id": "PMID:16848760", "title": "Man2C1, an alpha-mannosidase, is involved in the trimming of free oligosaccharides in the cytosol", "journal": "Biochem. J.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16848760/" } ], "x": 3321.3333333333335, "y": 839.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6799581", "label": "MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "oligosaccharide-recovery" ], "compartment": "lysosomal lumen", "compartment_ids": [ "GO:0043202" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-6799581", "summary": "MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man is a Reactome release 97 curated reaction event. The source record connects alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc, H2O to GlcNAc:Man, Man in lysosomal lumen.", "equation": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc + H2O → GlcNAc:Man + 2 Man", "inputs": [ { "node_id": "R-ALL-6799577", "reactome_id": "R-ALL-6799577", "label": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:91279", "formula": "C26H45NO21", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1605715", "reactome_id": "R-ALL-1605715", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-8853719", "reactome_id": "R-ALL-8853719", "label": "GlcNAc:Man", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:71553", "formula": "C14H25NO11", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-6799594", "reactome_id": "R-ALL-6799594", "label": "Man", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:4208", "formula": "C6H12O6", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "mannan endo-1,6-alpha-mannosidase activity of MAN2B2:Zn2+ [lysosomal lumen]", "activity_id": "6799586", "ec_numbers": [ "3.2.1.101" ], "accessions": null } ], "citations": [ { "id": "PMID:19722277", "title": "Human lysosomal alpha-mannosidases exhibit different inhibition and metal binding properties", "journal": "Protein Sci.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19722277/" }, { "id": "PMID:16115860", "title": "Characterization of a human core-specific lysosomal {alpha}1,6-mannosidase involved in N-glycan catabolism", "journal": "J. Biol. Chem.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16115860/" } ], "x": 3600, "y": 839.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6799604", "label": "GNPDA1,2 hexamers deaminate GlcN6P to Fru(6)P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:12173" ], "source_url": "https://reactome.org/content/detail/R-HSA-6799604", "summary": "GNPDA1,2 hexamers deaminate GlcN6P to Fru(6)P is a Reactome release 97 curated reaction event. The source record connects GlcN6P, H2O to Fru(6)P, NH4+ in cytosol and cross-references RHEA:12173.", "equation": "GlcN6P + H2O → Fru(6)P + NH4+", "inputs": [ { "node_id": "R-ALL-449701", "reactome_id": "R-ALL-449701", "label": "GlcN6P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:75989", "formula": "C6H13NO8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glucosamine-6-phosphate deaminase activity of GNPDA1,2 hexamers [cytosol]", "activity_id": "6799599", "ec_numbers": [ "3.5.99.6" ], "accessions": null } ], "citations": [ { "id": "PMID:9438414", "title": "Molecularly cloned mammalian glucosamine-6-phosphate deaminase localizes to transporting epithelium and lacks oscillin activity", "journal": "FASEB J.", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9438414/" }, { "id": "PMID:12616532", "title": "Cloning and functional characterization of GNPI2, a novel human homolog of glucosamine-6-phosphate isomerase/oscillin", "journal": "J. Cell. Biochem.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12616532/" }, { "id": "PMID:12965206", "title": "Two mammalian glucosamine-6-phosphate deaminases: a structural and genetic study", "journal": "FEBS Lett.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12965206/" } ], "x": 868.6666666666667, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6800334", "label": "MOGAT1,2,3 transfer acyl group from acyl-CoA to 2-acylglycerol to form DAG", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:32948" ], "source_url": "https://reactome.org/content/detail/R-HSA-6800334", "summary": "MOGAT1,2,3 transfer acyl group from acyl-CoA to 2-acylglycerol to form DAG is a Reactome release 97 curated reaction event. The source record connects 2AG, acyl-CoA to DAG, CoA-SH in endoplasmic reticulum membrane · cytosol and cross-references RHEA:32948.", "equation": "2AG + acyl-CoA → DAG + CoA-SH", "inputs": [ { "node_id": "R-ALL-6800352", "reactome_id": "R-ALL-6800352", "label": "2AG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17389", "formula": "C4H7O4R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1500578", "reactome_id": "R-ALL-1500578", "label": "acyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58342", "formula": "C22H31N7O17P3SR", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76103", "reactome_id": "R-ALL-76103", "label": "DAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "2-acylglycerol O-acyltransferase activity of MOGAT1,2,3 [endoplasmic reticulum membrane]", "activity_id": "6800350", "ec_numbers": [ "2.3.1.22" ], "accessions": null } ], "citations": [ { "id": "PMID:12618427", "title": "Identification of acyl coenzyme A:monoacylglycerol acyltransferase 3, an intestinal specific enzyme implicated in dietary fat absorption", "journal": "J. Biol. Chem.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12618427/" }, { "id": "PMID:12824082", "title": "Human intestinal monoacylglycerol acyltransferase: differential features in tissue expression and activity", "journal": "Am. J. Physiol. Endocrinol. Metab.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12824082/" }, { "id": "PMID:14970677", "title": "Genomic organization of the DGAT2/MOGAT gene family in cattle (Bos taurus) and other mammals", "journal": "Cytogenet. Genome Res.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14970677/" }, { "id": "PMID:25838202", "title": "Monoacylglycerol O-acyltransferase 1 is regulated by peroxisome proliferator-activated receptor γ in human hepatocytes and increases lipid accumulation", "journal": "Biochem. Biophys. Res. Commun.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25838202/" } ], "x": 3878.666666666667, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6801328", "label": "MTHFD2, D2L oxidise 5,10-methylene-THF to 5,10-methenyl-THF", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:22893" ], "source_url": "https://reactome.org/content/detail/R-HSA-6801328", "summary": "MTHFD2, D2L oxidise 5,10-methylene-THF to 5,10-methenyl-THF is a Reactome release 97 curated reaction event. The source record connects 5,10-methylene-THF, NAD+ to 5,10-methenyl-THF, NADH in mitochondrial matrix and cross-references RHEA:22893.", "equation": "5,10-methylene-THF + NAD+ → 5,10-methenyl-THF + NADH", "inputs": [ { "node_id": "R-ALL-5693984", "reactome_id": "R-ALL-5693984", "label": "5,10-methylene-THF", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15636", "formula": "C20H21N7O6", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6801458", "reactome_id": "R-ALL-6801458", "label": "5,10-methenyl-THF", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57455", "formula": "C20H20N7O6", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "methenyltetrahydrofolate cyclohydrolase activity of MTHFD2, D2L [mitochondrial matrix]", "activity_id": "6801460", "ec_numbers": [ "3.5.4.9" ], "accessions": null } ], "citations": [ { "id": "PMID:24733394", "title": "Mitochondrial MTHFD2L is a dual redox cofactor-specific methylenetetrahydrofolate dehydrogenase/methenyltetrahydrofolate cyclohydrolase expressed in both adult and embryonic tissues", "journal": "J. Biol. Chem.", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24733394/" }, { "id": "PMID:21163947", "title": "Mammalian MTHFD2L encodes a mitochondrial methylenetetrahydrofolate dehydrogenase isozyme expressed in adult tissues", "journal": "J. Biol. Chem.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21163947/" }, { "id": "PMID:8218174", "title": "NAD-dependent methylenetetrahydrofolate dehydrogenase-methenyltetrahydrofolate cyclohydrolase is the mammalian homolog of the mitochondrial enzyme encoded by the yeast MIS1 gene", "journal": "Biochemistry", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8218174/" } ], "x": 4451.333333333333, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6803255", "label": "HCOOH translocated from mitochondrial matrix to cytosol", "schema_class": "BlackBoxEvent", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "cytosol · mitochondrial matrix", "compartment_ids": [ "GO:0005829", "GO:0005759" ], "rhea_ids": [ "RHEA:29679" ], "source_url": "https://reactome.org/content/detail/R-HSA-6803255", "summary": "HCOOH translocated from mitochondrial matrix to cytosol is a Reactome release 97 curated black-box event whose internal chemistry is not expanded by the source record. The source record connects HCOOH to HCOOH in cytosol · mitochondrial matrix and cross-references RHEA:29679.", "equation": "HCOOH → HCOOH", "inputs": [ { "node_id": "R-ALL-6801451", "reactome_id": "R-ALL-6801451", "label": "HCOOH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15740", "formula": "CHO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29460", "reactome_id": "R-ALL-29460", "label": "HCOOH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15740", "formula": "CHO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [], "citations": [ { "id": "PMID:20645850", "title": "Compartmentalization of Mammalian folate-mediated one-carbon metabolism", "journal": "Annu. Rev. Nutr.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20645850/" } ], "x": 4730, "y": 3046.125, "lod": "pathway", "collapsed": true, "transport": true }, { "id": "R-HSA-6807055", "label": "DHCR7 reduces 7-dehydroCHOL to CHOL", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "cholesterol", "steroids" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:23984", "RHEA:23986" ], "source_url": "https://reactome.org/content/detail/R-HSA-6807055", "summary": "DHCR7 reduces 7-dehydroCHOL to CHOL is a Reactome release 97 curated reaction event. The source record connects 7-dehydroCHOL, NADPH, H+ to CHOL, NADP+ in endoplasmic reticulum membrane · cytosol and cross-references RHEA:23984, RHEA:23986.", "equation": "7-dehydroCHOL + NADPH + H+ → CHOL + NADP+", "inputs": [ { "node_id": "R-ALL-6807057", "reactome_id": "R-ALL-6807057", "label": "7-dehydroCHOL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17759", "formula": "C27H44O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-193384", "reactome_id": "R-ALL-193384", "label": "CHOL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16113", "formula": "C27H46O", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "7-dehydrocholesterol reductase activity of DHCR7 [endoplasmic reticulum membrane]", "activity_id": "196403", "ec_numbers": [ "1.3.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:14404284", "title": "Preputial gland tumor sterols. 3. A metabolic pathway from lanosterol to cholesterol", "journal": "J. Biol. Chem.", "year": 1960, "url": "https://pubmed.ncbi.nlm.nih.gov/14404284/" }, { "id": "PMID:26114596", "title": "Flux analysis of cholesterol biosynthesis in vivo reveals multiple tissue and cell-type specific pathways", "journal": "Elife", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/26114596/" }, { "id": "PMID:9465114", "title": "Molecular cloning and expression of the human delta7-sterol reductase", "journal": "Proc Natl Acad Sci U S A", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9465114/" } ], "x": 2938.666666666667, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6807826", "label": "LDHAL6B reduces PYR to LACT", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:23446", "RHEA:23447" ], "source_url": "https://reactome.org/content/detail/R-HSA-6807826", "summary": "LDHAL6B reduces PYR to LACT is a Reactome release 97 curated reaction event. The source record connects PYR, NADH, H+ to LACT, NAD+ in mitochondrial matrix and cross-references RHEA:23446, RHEA:23447.", "equation": "PYR + NADH + H+ → LACT + NAD+", "inputs": [ { "node_id": "R-ALL-113557", "reactome_id": "R-ALL-113557", "label": "PYR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6807616", "reactome_id": "R-ALL-6807616", "label": "LACT", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:422", "formula": "C3H6O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-lactate dehydrogenase (NAD+) activity of LDHAL6B [mitochondrial matrix]", "activity_id": "6807609", "ec_numbers": [ "1.1.1.27" ], "accessions": null } ], "citations": [ { "id": "PMID:19679512", "title": "Computational analyses of mammalian lactate dehydrogenases: human, mouse, opossum and platypus LDHs", "journal": "Comput Biol Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19679512/" }, { "id": "PMID:15870898", "title": "Cloning and characterization of a novel intronless lactate dehydrogenase gene in human testis", "journal": "Int. J. Mol. Med.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15870898/" } ], "x": 4730, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6808464", "label": "ALDH3B2 oxidises HXAL to PALM", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "lipid droplet · cytosol", "compartment_ids": [ "GO:0005811", "GO:0005829" ], "rhea_ids": [ "RHEA:33739" ], "source_url": "https://reactome.org/content/detail/R-HSA-6808464", "summary": "ALDH3B2 oxidises HXAL to PALM is a Reactome release 97 curated reaction event. The source record connects HXAL, H2O, NAD+ to PALM, NADH, H+ in lipid droplet · cytosol and cross-references RHEA:33739.", "equation": "HXAL + H2O + NAD+ → PALM + NADH + H+", "inputs": [ { "node_id": "R-ALL-6808468", "reactome_id": "R-ALL-6808468", "label": "HXAL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17600", "formula": "C16H32O", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2453691", "reactome_id": "R-ALL-2453691", "label": "PALM", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:7896", "formula": "C16H31O2", "compartment": "lipid droplet", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aldehyde dehydrogenase [NAD(P)+] activity of ALDH3B2 [lipid droplet]", "activity_id": "6808465", "ec_numbers": [ "1.2.1.5" ], "accessions": null } ], "citations": [ { "id": "PMID:23721920", "title": "Substrate specificity, plasma membrane localization, and lipid modification of the aldehyde dehydrogenase ALDH3B1", "journal": "Biochim. 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The source record connects 10-formyl-THFPG, NADP+, H2O to THFPG, NADPH, H+ in mitochondrial matrix.", "equation": "10-formyl-THFPG + NADP+ + H2O → THFPG + NADPH + H+ + CO2", "inputs": [ { "node_id": "R-ALL-6808499", "reactome_id": "R-ALL-6808499", "label": "10-formyl-THFPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28010", "formula": "(C5H7NO3)n.C25H30N8O10", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-200679", "reactome_id": "R-ALL-200679", "label": "THFPG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28624", "formula": "(C5H7NO3)n.C19H23N7O6", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "aldehyde dehydrogenase (NAD+) activity of ADH1L2 tetramer [mitochondrial matrix]", "activity_id": "6808492", "ec_numbers": [ "1.2.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:20498374", "title": "ALDH1L2 is the mitochondrial homolog of 10-formyltetrahydrofolate dehydrogenase", "journal": "J. Biol. Chem.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20498374/" }, { "id": "PMID:21238436", "title": "Enzymatic properties of ALDH1L2, a mitochondrial 10-formyltetrahydrofolate dehydrogenase", "journal": "Chem. Biol. Interact.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21238436/" } ], "x": 5008.666666666667, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6808496", "label": "ALDH1L1 dehydrogenates 10-formyl-THFPG to THFPG", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-6808496", "summary": "ALDH1L1 dehydrogenates 10-formyl-THFPG to THFPG is a Reactome release 97 curated reaction event. The source record connects 10-formyl-THFPG, NADP+, H2O to THFPG, NADPH, H+ in cytosol.", "equation": "10-formyl-THFPG + NADP+ + H2O → THFPG + NADPH + H+ + CO2", "inputs": [ { "node_id": "R-ALL-111353", "reactome_id": "R-ALL-111353", "label": "10-formyl-THFPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28010", "formula": "(C5H7NO3)n.C25H30N8O10", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-197969", "reactome_id": "R-ALL-197969", "label": "THFPG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28624", "formula": "(C5H7NO3)n.C19H23N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "aldehyde dehydrogenase (NAD+) activity of ALDH1L1 tetramer [cytosol]", "activity_id": "6808489", "ec_numbers": [ "1.2.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:20498374", "title": "ALDH1L2 is the mitochondrial homolog of 10-formyltetrahydrofolate dehydrogenase", "journal": "J. Biol. Chem.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20498374/" }, { "id": "PMID:21238436", "title": "Enzymatic properties of ALDH1L2, a mitochondrial 10-formyltetrahydrofolate dehydrogenase", "journal": "Chem. Biol. Interact.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21238436/" } ], "x": 4451.333333333333, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6809287", "label": "NUDT12 hydrolyses NADH to NMNH", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:48869" ], "source_url": "https://reactome.org/content/detail/R-HSA-6809287", "summary": "NUDT12 hydrolyses NADH to NMNH is a Reactome release 97 curated reaction event. The source record connects NADH, H2O to NMNH, H+, AMP in peroxisomal matrix and cross-references RHEA:48869.", "equation": "NADH + H2O → NMNH + 2 H+ + AMP", "inputs": [ { "node_id": "R-ALL-192305", "reactome_id": "R-ALL-192305", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6809289", "reactome_id": "R-ALL-6809289", "label": "NMNH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:90832", "formula": "C11H15N2O8P", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-193465", "reactome_id": "R-ALL-193465", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-389620", "reactome_id": "R-ALL-389620", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "NADH pyrophosphatase activity of NUDT12 [peroxisomal matrix]", "activity_id": "6809293", "ec_numbers": [ "3.6.1.22" ], "accessions": null } ], "citations": [ { "id": "PMID:12790796", "title": "Mammalian NADH diphosphatases of the Nudix family: cloning and characterization of the human peroxisomal NUDT12 protein", "journal": "Biochem. J.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12790796/" } ], "x": 5948.666666666667, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6810076", "label": "DDO oxidizes D-Asp to OA", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "glycine-glyoxylate" ], "compartment": "peroxisomal matrix", "compartment_ids": [ "GO:0005782" ], "rhea_ids": [ "RHEA:12513" ], "source_url": "https://reactome.org/content/detail/R-HSA-6810076", "summary": "DDO oxidizes D-Asp to OA is a Reactome release 97 curated reaction event. The source record connects D-Asp, H2O, O2 to OA, NH4+, H2O2 in peroxisomal matrix and cross-references RHEA:12513.", "equation": "D-Asp + H2O + O2 → OA + NH4+ + H2O2", "inputs": [ { "node_id": "R-ALL-6810061", "reactome_id": "R-ALL-6810061", "label": "D-Asp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29990", "formula": "C4H6NO4", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-141343", "reactome_id": "R-ALL-141343", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113535", "reactome_id": "R-ALL-113535", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-6810070", "reactome_id": "R-ALL-6810070", "label": "OA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-389843", "reactome_id": "R-ALL-389843", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113575", "reactome_id": "R-ALL-113575", "label": "H2O2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16240", "formula": "H2O2", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "D-aspartate oxidase activity of DDO:FAD [peroxisomal matrix]", "activity_id": "6810051", "ec_numbers": [ "1.4.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:9820813", "title": "C-terminal tripeptide Ser-Asn-Leu (SNL) of human D-aspartate oxidase is a functional peroxisome-targeting signal", "journal": "Biochem. J.", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9820813/" }, { "id": "PMID:25747990", "title": "Characterization of the enzymatic and structural properties of human D-aspartate oxidase and comparison with those of the rat and mouse enzymes", "journal": "Biol. Pharm. Bull.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25747990/" }, { "id": "PMID:20603179", "title": "Thiolactomycin inhibits D-aspartate oxidase: a novel approach to probing the active site environment", "journal": "Biochimie", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20603179/" }, { "id": "PMID:1601857", "title": "The primary structure of the flavoprotein D-aspartate oxidase from beef kidney", "journal": "J. Biol. Chem.", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1601857/" }, { "id": "PMID:9163533", "title": "Structural and functional characterization of the human brain D-aspartate oxidase", "journal": "J. Biochem.", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9163533/" } ], "x": 868.6666666666667, "y": 3160.5555555555557, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-6813749", "label": "ALDH1A1 oxidises GA to DGA", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "fructose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:30784" ], "source_url": "https://reactome.org/content/detail/R-HSA-6813749", "summary": "ALDH1A1 oxidises GA to DGA is a Reactome release 97 curated reaction event. The source record connects GA, NAD+, H2O to DGA, NADH, H+ in cytosol and cross-references RHEA:30784.", "equation": "GA + NAD+ + H2O → DGA + NADH + 2 H+", "inputs": [ { "node_id": "R-ALL-30393", "reactome_id": "R-ALL-30393", "label": "GA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17378", "formula": "C3H6O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6799497", "reactome_id": "R-ALL-6799497", "label": "DGA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16659", "formula": "C3H5O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aldehyde dehydrogenase (NAD+) activity of ALDH1A1 tetramer [cytosol]", "activity_id": "71690", "ec_numbers": [ "1.2.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:23444097", "title": "New insights into the half-of-the-sites reactivity of human aldehyde dehydrogenase 1A1", "journal": "Proteins", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23444097/" } ], "x": 2381.3333333333335, "y": 327.8333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70241", "label": "PCK1 phosphorylates OA to yield PEP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10389" ], "source_url": "https://reactome.org/content/detail/R-HSA-70241", "summary": "PCK1 phosphorylates OA to yield PEP is a Reactome release 97 curated reaction event. The source record connects OA, GTP to PEP, CO2, GDP in cytosol and cross-references RHEA:10389.", "equation": "OA + GTP → PEP + CO2 + GDP", "inputs": [ { "node_id": "R-ALL-76213", "reactome_id": "R-ALL-76213", "label": "OA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29438", "reactome_id": "R-ALL-29438", "label": "GTP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:37565", "formula": "C10H12N5O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29492", "reactome_id": "R-ALL-29492", "label": "PEP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58702", "formula": "C3H2O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29420", "reactome_id": "R-ALL-29420", "label": "GDP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58189", "formula": "C10H12N5O11P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoenolpyruvate carboxykinase (GTP) activity of PCK1 [cytosol]", "activity_id": "70496", "ec_numbers": [ "4.1.1.32" ], "accessions": null } ], "citations": [ { "id": "PMID:11851336", "title": "Crystal structure of human cytosolic phosphoenolpyruvate carboxykinase reveals a new GTP-binding site", "journal": "J Mol Biol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11851336/" } ], "x": 1251.3333333333333, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70262", "label": "PFKFKB dimer dephosphorylates fructose 2,6-bisphosphate", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17290" ], "source_url": "https://reactome.org/content/detail/R-HSA-70262", "summary": "PFKFKB dimer dephosphorylates fructose 2,6-bisphosphate is a Reactome release 97 curated reaction event. The source record connects D-Fructose 2,6-bisphosphate, H2O to Fru(6)P, Pi in cytosol and cross-references RHEA:17290.", "equation": "D-Fructose 2,6-bisphosphate + H2O → Fru(6)P + Pi", "inputs": [ { "node_id": "R-ALL-30533", "reactome_id": "R-ALL-30533", "label": "D-Fructose 2,6-bisphosphate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58579", "formula": "C6H10O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "fructose-2,6-bisphosphate 2-phosphatase activity of p-PFKFB1 dimer [cytosol]", "activity_id": "177980", "ec_numbers": [ "3.1.3.46" ], "accessions": null } ], "citations": [ { "id": "PMID:7574501", "title": "6-Phosphofructo-2-kinase/fructose-2,6-bisphosphatase: a metabolic signaling enzyme.", "journal": "Annu Rev Biochem", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7574501/" } ], "x": 311.33333333333337, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70286", "label": "UTP + D-glucose 1-phosphate =\u003e pyrophosphate + UDP-glucose", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "biological-oxidations", "glycogen", "phase-two" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19890" ], "source_url": "https://reactome.org/content/detail/R-HSA-70286", "summary": "UTP + D-glucose 1-phosphate =\u003e pyrophosphate + UDP-glucose is a Reactome release 97 curated reaction event. The source record connects UTP, G1P, H+ to PPi, UDP-Glc in cytosol and cross-references RHEA:19890.", "equation": "UTP + G1P + H+ → PPi + UDP-Glc", "inputs": [ { "node_id": "R-ALL-29494", "reactome_id": "R-ALL-29494", "label": "UTP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46398", "formula": "C9H11N2O15P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29548", "reactome_id": "R-ALL-29548", "label": "G1P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58601", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "UTP:glucose-1-phosphate uridylyltransferase activity of 8xUGP2 [cytosol]", "activity_id": "70285", "ec_numbers": [ "2.7.7.9" ], "accessions": null } ], "citations": [ { "id": "PMID:8631325", "title": "Sequence differences between human muscle and liver cDNAs for UDPglucose pyrophosphorylase and kinetic properties of the recombinant enzymes expressed in Escherichia coli.", "journal": "Eur J Biochem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8631325/" }, { "id": "PMID:5427280", "title": "Uridine diphosphate glucose pyrophosphorylase. IV. Crystallization and properties of the enzyme from human liver", "journal": "J Biol Chem", "year": 1970, "url": "https://pubmed.ncbi.nlm.nih.gov/5427280/" }, { "id": "PMID:5348606", "title": "Uridine diphosphate glucose pyrophosphorylase. II", "journal": "J Biol Chem", "year": 1969, "url": "https://pubmed.ncbi.nlm.nih.gov/5348606/" } ], "x": 1251.3333333333333, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70333", "label": "KHK dimer phosphorylates Fru to Fru 1-P", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "fructose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18146" ], "source_url": "https://reactome.org/content/detail/R-HSA-70333", "summary": "KHK dimer phosphorylates Fru to Fru 1-P is a Reactome release 97 curated reaction event. The source record connects ATP, beta-D-Fru to ADP, beta-D-Fru 1-P, H+ in cytosol and cross-references RHEA:18146.", "equation": "ATP + beta-D-Fru → ADP + beta-D-Fru 1-P + H+", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9832977", "reactome_id": "R-ALL-9832977", "label": "beta-D-Fru", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28645", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9832976", "reactome_id": "R-ALL-9832976", "label": "beta-D-Fru 1-P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:138881", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ketohexokinase activity of KHK dimer [cytosol]", "activity_id": "70330", "ec_numbers": [ "2.7.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:12941785", "title": "Properties of normal and mutant recombinant human ketohexokinases and implications for the pathogenesis of essential fructosuria", "journal": "Diabetes", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12941785/" }, { "id": "PMID:7833921", "title": "Molecular basis of essential fructosuria: molecular cloning and mutational analysis of human ketohexokinase (fructokinase)", "journal": "Hum Mol Genet", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7833921/" }, { "id": "PMID:20841500", "title": "Both isoforms of ketohexokinase are dispensable for normal growth and development", "journal": "Physiol. Genomics", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20841500/" }, { "id": "PMID:16266770", "title": "Genes required for fructose metabolism are expressed in Purkinje cells in the cerebellum", "journal": "Brain Res. Mol. Brain Res.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16266770/" }, { "id": "PMID:22371574", "title": "Opposing effects of fructokinase C and A isoforms on fructose-induced metabolic syndrome in mice", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22371574/" }, { "id": "PMID:19237742", "title": "Structures of alternatively spliced isoforms of human ketohexokinase", "journal": "Acta Crystallogr D Biol Crystallogr", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19237742/" } ], "x": 2660, "y": 457.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70342", "label": "ALDOB tetramer cleaves Fru-1-P to GA and DHAP", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "fructose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:30852" ], "source_url": "https://reactome.org/content/detail/R-HSA-70342", "summary": "ALDOB tetramer cleaves Fru-1-P to GA and DHAP is a Reactome release 97 curated reaction event. The source record connects beta-D-Fru 1-P to GA, DHAP in cytosol and cross-references RHEA:30852.", "equation": "beta-D-Fru 1-P → GA + DHAP", "inputs": [ { "node_id": "R-ALL-9832976", "reactome_id": "R-ALL-9832976", "label": "beta-D-Fru 1-P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:138881", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-30393", "reactome_id": "R-ALL-30393", "label": "GA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17378", "formula": "C3H6O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-75970", "reactome_id": "R-ALL-75970", "label": "DHAP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57642", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "fructose-1-phosphate aldolase activity of ALDOB tetramer [cytosol]", "activity_id": "469725", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:9610797", "title": "Hereditary fructose intolerance", "journal": "J. Med. Genet.", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9610797/" }, { "id": "PMID:11679716", "title": "The structure of human liver fructose-1,6-bisphosphate aldolase", "journal": "Acta Crystallogr D Biol Crystallogr", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11679716/" }, { "id": "PMID:13093749", "title": "[The metabolism of fructose-1-phosphate in the liver.]", "journal": "Biochim Biophys Acta", "year": 1953, "url": "https://pubmed.ncbi.nlm.nih.gov/13093749/" }, { "id": "PMID:5777313", "title": "Distribution of fructose diphosphate aldolase variants in biological systems", "journal": "Biochemistry", "year": 1969, "url": "https://pubmed.ncbi.nlm.nih.gov/5777313/" }, { "id": "PMID:5353106", "title": "Isolation of fructose diphosphate aldolases A, B, and C", "journal": "Biochemistry", "year": 1969, "url": "https://pubmed.ncbi.nlm.nih.gov/5353106/" }, { "id": "PMID:805723", "title": "Kinetic and immunological abnormalities of aldolase B in hereditary fructose intolerance", "journal": "Biochem. Soc. Trans.", "year": 1975, "url": "https://pubmed.ncbi.nlm.nih.gov/805723/" } ], "x": 2381.3333333333335, "y": 587.1666666666666, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70349", "label": "DAK dimer phosphorylates D-glyceraldehyde to form D-glyceraldehyde 3-phosphate", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "fructose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13942" ], "source_url": "https://reactome.org/content/detail/R-HSA-70349", "summary": "DAK dimer phosphorylates D-glyceraldehyde to form D-glyceraldehyde 3-phosphate is a Reactome release 97 curated reaction event. The source record connects ATP, GA to ADP, GA3P in cytosol and cross-references RHEA:13942.", "equation": "ATP + GA → ADP + GA3P", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-30393", "reactome_id": "R-ALL-30393", "label": "GA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17378", "formula": "C3H6O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "triokinase activity of TKFC:2Mg2+ dimer [cytosol]", "activity_id": "70346", "ec_numbers": [ "2.7.1.28" ], "accessions": null } ], "citations": [ { "id": "PMID:4688871", "title": "Dihydroxyacetone metabolism by human erythrocytes: demonstration of triokinase activity and its characterization", "journal": "Blood", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4688871/" }, { "id": "PMID:16289032", "title": "Identification of human and rat FAD-AMP lyase (cyclic FMN forming) as ATP-dependent dihydroxyacetone kinases", "journal": "Biochem. Biophys. Res. Commun.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16289032/" }, { "id": "PMID:13093749", "title": "[The metabolism of fructose-1-phosphate in the liver.]", "journal": "Biochim Biophys Acta", "year": 1953, "url": "https://pubmed.ncbi.nlm.nih.gov/13093749/" }, { "id": "PMID:22098443", "title": "Liver protein profiling in chronic hepatitis C: identification of potential predictive markers for interferon therapy outcome", "journal": "J. Proteome Res.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22098443/" }, { "id": "PMID:24569995", "title": "Bifunctional homodimeric triokinase/FMN cyclase: contribution of protein domains to the activities of the human enzyme and molecular dynamics simulation of domain movements", "journal": "J. Biol. Chem.", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24569995/" }, { "id": "PMID:24289155", "title": "Serum dihydroxyacetone kinase peptide m/z 520.3 as predictor of disease severity in patients with compensated chronic hepatitis B", "journal": "J Transl Med", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/24289155/" } ], "x": 2938.666666666667, "y": 457.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70355", "label": "GALK1 phosphorylates Gal to Gal1P", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "galactose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13554", "RHEA:13553" ], "source_url": "https://reactome.org/content/detail/R-HSA-70355", "summary": "GALK1 phosphorylates Gal to Gal1P is a Reactome release 97 curated reaction event. The source record connects ATP, alpha-D-Gal to ADP, Gal1P, H+ in cytosol and cross-references RHEA:13554, RHEA:13553.", "equation": "ATP + alpha-D-Gal → ADP + Gal1P + H+", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9931851", "reactome_id": "R-ALL-9931851", "label": "alpha-D-Gal", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28061", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-30170", "reactome_id": "R-ALL-30170", "label": "Gal1P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17973", "formula": "C6H13O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "galactokinase activity of GALK1 [cytosol]", "activity_id": "70354", "ec_numbers": [ "2.7.1.6" ], "accessions": null } ], "citations": [ { "id": "PMID:7542884", "title": "Comparison of the enzymatic activities of human galactokinase GALK1 and a related human galactokinase protein GK2", "journal": "Biochem Biophys Res Commun", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7542884/" } ], "x": 3321.3333333333335, "y": 360.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70361", "label": "GALT transfers UMP from UDP-Glc to Gal1P to form UDP-Gal", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "galactose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13990" ], "source_url": "https://reactome.org/content/detail/R-HSA-70361", "summary": "GALT transfers UMP from UDP-Glc to Gal1P to form UDP-Gal is a Reactome release 97 curated reaction event. The source record connects UDP-Glc, Gal1P to G1P, UDP-Gal in cytosol and cross-references RHEA:13990.", "equation": "UDP-Glc + Gal1P → G1P + UDP-Gal", "inputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-30170", "reactome_id": "R-ALL-30170", "label": "Gal1P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17973", "formula": "C6H13O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29548", "reactome_id": "R-ALL-29548", "label": "G1P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58601", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29452", "reactome_id": "R-ALL-29452", "label": "UDP-Gal", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:66914", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "UDP-glucose:hexose-1-phosphate uridylyltransferase activity of GALT [cytosol]", "activity_id": "70360", "ec_numbers": [ "2.7.7.12" ], "accessions": null } ], "citations": [ { "id": "PMID:1897530", "title": "Molecular characterization of two galactosemia mutations: correlation of mutations with highly conserved domains in galactose-1-phosphate uridyl transferase", "journal": "Am J Hum Genet", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1897530/" }, { "id": "PMID:2011574", "title": "Molecular basis of galactosemia: mutations and polymorphisms in the gene encoding human galactose-1-phosphate uridylyltransferase", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/2011574/" } ], "x": 3878.666666666667, "y": 360.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70369", "label": "GALE:NAD+ dimer reversibly epimerises UDP-Gal to UDP-Glc", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "galactose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:22170" ], "source_url": "https://reactome.org/content/detail/R-HSA-70369", "summary": "GALE:NAD+ dimer reversibly epimerises UDP-Gal to UDP-Glc is a Reactome release 97 curated reaction event. The source record connects UDP-Gal to UDP-Glc in cytosol and cross-references RHEA:22170.", "equation": "UDP-Gal → UDP-Glc", "inputs": [ { "node_id": "R-ALL-29452", "reactome_id": "R-ALL-29452", "label": "UDP-Gal", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:66914", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29410", "reactome_id": "R-ALL-29410", "label": "UDP-Glc", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58885", "formula": "C15H22N2O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "UDP-glucose 4-epimerase activity of GALE:NAD+ dimer [cytosol]", "activity_id": "70368", "ec_numbers": [ "5.1.3.2" ], "accessions": null } ], "citations": [ { "id": "PMID:15175331", "title": "Determinants of function and substrate specificity in human UDP-galactose 4'-epimerase", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15175331/" }, { "id": "PMID:10801319", "title": "Crystallographic evidence for Tyr 157 functioning as the active site base in human UDP-galactose 4-epimerase", "journal": "Biochemistry", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10801319/" } ], "x": 3321.3333333333335, "y": 554.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70449", "label": "GADPH tetramer dehydrogenates GA3P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10301" ], "source_url": "https://reactome.org/content/detail/R-HSA-70449", "summary": "GADPH tetramer dehydrogenates GA3P is a Reactome release 97 curated reaction event. The source record connects GA3P, Pi, NAD+ to 1,3BPG, NADH, H+ in cytosol and cross-references RHEA:10301.", "equation": "GA3P + Pi + NAD+ → 1,3BPG + NADH + H+", "inputs": [ { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29800", "reactome_id": "R-ALL-29800", "label": "1,3BPG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57604", "formula": "C3H4O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glyceraldehyde-3-phosphate dehydrogenase (NAD+) (phosphorylating) activity of GAPDH tetramers [cytosol]", "activity_id": "70448", "ec_numbers": [ "1.2.1.12" ], "accessions": null } ], "citations": [ { "id": "PMID:2793178", "title": "Members of the human glyceraldehyde-3-phosphate dehydrogenase-related gene family map to dispersed chromosomal locations.", "journal": "Genomics", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2793178/" }, { "id": "PMID:18910716", "title": "Crystalline D-glyceraldehyde-3-phosphate dehydrogenase from rabbit muscle", "journal": "J Biol Chem", "year": 1948, "url": "https://pubmed.ncbi.nlm.nih.gov/18910716/" }, { "id": "PMID:183598", "title": "Isozymes of glyceraldehyde-3-phosphate dehydrogenase in man and other mammals.", "journal": "Ann Hum Genet", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/183598/" }, { "id": "PMID:3170585", "title": "Isolation and complete sequence of a functional human glyceraldehyde-3-phosphate dehydrogenase gene", "journal": "J Biol Chem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3170585/" }, { "id": "PMID:7144574", "title": "Glyceraldehyde-3-phosphate dehydrogenase from human tissues.", "journal": "Methods Enzymol", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/7144574/" }, { "id": "PMID:10714828", "title": "Human glyceraldehyde 3-phosphate dehydrogenase-2 gene is expressed specifically in spermatogenic cells.", "journal": "J Androl", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10714828/" } ], "x": 311.33333333333337, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70454", "label": "TPI1 dimer isomerizes DHAP to GA3P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18587" ], "source_url": "https://reactome.org/content/detail/R-HSA-70454", "summary": "TPI1 dimer isomerizes DHAP to GA3P is a Reactome release 97 curated reaction event. The source record connects DHAP to GA3P in cytosol and cross-references RHEA:18587.", "equation": "DHAP → GA3P", "inputs": [ { "node_id": "R-ALL-75970", "reactome_id": "R-ALL-75970", "label": "DHAP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57642", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "triose-phosphate isomerase activity of TPI1 dimer [cytosol]", "activity_id": "70453", "ec_numbers": [ "5.3.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:6434534", "title": "Primary structure of human triosephosphate isomerase", "journal": "J Biol Chem", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6434534/" }, { "id": "PMID:16511037", "title": "Structure of a high-resolution crystal form of human triosephosphate isomerase: improvement of crystals using the gel-tube method", "journal": "Acta Crystallogr Sect F Struct Biol Cryst Commun", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16511037/" } ], "x": 868.6666666666667, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70467", "label": "PFK tetramer phosphorylates Fru(6)P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:16110" ], "source_url": "https://reactome.org/content/detail/R-HSA-70467", "summary": "PFK tetramer phosphorylates Fru(6)P is a Reactome release 97 curated reaction event. The source record connects Fru(6)P, ATP to F1,6PP, ADP, H+ in cytosol and cross-references RHEA:16110.", "equation": "Fru(6)P + ATP → F1,6PP + ADP + H+", "inputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-30014", "reactome_id": "R-ALL-30014", "label": "F1,6PP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:32966", "formula": "C6H10O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "6-phosphofructokinase activity of PFK tetramer [cytosol]", "activity_id": "70466", "ec_numbers": [ "2.7.1.11" ], "accessions": null } ], "citations": [ { "id": "PMID:8117307", "title": "Cloning of a complete protein-coding sequence of human platelet-type phosphofructokinase isozyme from pancreatic islet.", "journal": "Biochem Biophys Res Commun", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8117307/" }, { "id": "PMID:7825568", "title": "Functional expression of human mutant phosphofructokinase in yeast: genetic defects in French Canadian and Swiss patients with phosphofructokinase deficiency", "journal": "Am J Hum Genet", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7825568/" }, { "id": "PMID:6451249", "title": "Isozymes of human phosphofructokinase in blood cells and cultured cell lines: molecular and genetic evidence for a trigenic system", "journal": "Blood", "year": 1981, "url": "https://pubmed.ncbi.nlm.nih.gov/6451249/" }, { "id": "PMID:6444721", "title": "Isozymes of human phosphofructokinase: identification and subunit structural characterization of a new system", "journal": "Proc Natl Acad Sci U S A", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6444721/" }, { "id": "PMID:2960695", "title": "Characterization of the enzymatic defect in late-onset muscle phosphofructokinase deficiency. New subtype of glycogen storage disease type VII.", "journal": "J Clin Invest", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/2960695/" } ], "x": 590, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70475", "label": "GPI dimer isomerizes Fru(6)P to G6P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11818" ], "source_url": "https://reactome.org/content/detail/R-HSA-70475", "summary": "GPI dimer isomerizes Fru(6)P to G6P is a Reactome release 97 curated reaction event. The source record connects Fru(6)P to G6P in cytosol and cross-references RHEA:11818.", "equation": "Fru(6)P → G6P", "inputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-30537", "reactome_id": "R-ALL-30537", "label": "G6P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58225", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glucose-6-phosphate isomerase activity of GPI dimer [cytosol]", "activity_id": "70470", "ec_numbers": [ "5.3.1.9" ], "accessions": null } ], "citations": [ { "id": "PMID:7989588", "title": "The characterization of gene mutations for human glucose phosphate isomerase deficiency associated with chronic hemolytic anemia", "journal": "J Clin Invest", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7989588/" }, { "id": "PMID:13538944", "title": "Enzymes of the human erythrocyte. IV. Phosphoglucose isomerase, purification and properties.", "journal": "J Biol Chem", "year": 1958, "url": "https://pubmed.ncbi.nlm.nih.gov/13538944/" } ], "x": 1808.6666666666667, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70479", "label": "FBP tetramers hydrolyze F1,6PP to Fru(6)P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11065" ], "source_url": "https://reactome.org/content/detail/R-HSA-70479", "summary": "FBP tetramers hydrolyze F1,6PP to Fru(6)P is a Reactome release 97 curated reaction event. The source record connects H2O, F1,6PP to Fru(6)P, Pi in cytosol and cross-references RHEA:11065.", "equation": "H2O + F1,6PP → Fru(6)P + Pi", "inputs": [ { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-30014", "reactome_id": "R-ALL-30014", "label": "F1,6PP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:32966", "formula": "C6H10O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "fructose 1,6-bisphosphate 1-phosphatase activity of FBP tetramer [cytosol]", "activity_id": "70478", "ec_numbers": [ "3.1.3.11" ], "accessions": null } ], "citations": [ { "id": "PMID:9382095", "title": "Identification of genetic mutations in Japanese patients with fructose-1,6-bisphosphatase deficiency", "journal": "Am J Hum Genet", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9382095/" }, { "id": "PMID:9678974", "title": "Isolation and characterization of an allelic cDNA for human muscle fructose-1,6-bisphosphatase", "journal": "Gene", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9678974/" } ], "x": 1530, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70481", "label": "TPI1 dimer isomerizes GA3P to DHAP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18586" ], "source_url": "https://reactome.org/content/detail/R-HSA-70481", "summary": "TPI1 dimer isomerizes GA3P to DHAP is a Reactome release 97 curated reaction event. The source record connects GA3P to DHAP in cytosol and cross-references RHEA:18586.", "equation": "GA3P → DHAP", "inputs": [ { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-75970", "reactome_id": "R-ALL-75970", "label": "DHAP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57642", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "triose-phosphate isomerase activity of TPI1 dimer [cytosol]", "activity_id": "70453", "ec_numbers": [ "5.3.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:8571957", "title": "Molecular analysis of a series of alleles in humans with reduced activity at the triosephosphate isomerase locus", "journal": "Am J Hum Genet", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8571957/" }, { "id": "PMID:6434534", "title": "Primary structure of human triosephosphate isomerase", "journal": "J Biol Chem", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6434534/" } ], "x": 1251.3333333333333, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70482", "label": "GAPDH tetramers reduce 1,3BPG to GA3P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10302" ], "source_url": "https://reactome.org/content/detail/R-HSA-70482", "summary": "GAPDH tetramers reduce 1,3BPG to GA3P is a Reactome release 97 curated reaction event. The source record connects 1,3BPG, NADH, H+ to GA3P, Pi, NAD+ in cytosol and cross-references RHEA:10302.", "equation": "1,3BPG + NADH + H+ → GA3P + Pi + NAD+", "inputs": [ { "node_id": "R-ALL-29800", "reactome_id": "R-ALL-29800", "label": "1,3BPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57604", "formula": "C3H4O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glyceraldehyde-3-phosphate dehydrogenase (NAD+) (phosphorylating) activity of GAPDH tetramers [cytosol]", "activity_id": "70448", "ec_numbers": [ "1.2.1.12" ], "accessions": null } ], "citations": [ { "id": "PMID:7144574", "title": "Glyceraldehyde-3-phosphate dehydrogenase from human tissues.", "journal": "Methods Enzymol", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/7144574/" }, { "id": "PMID:183598", "title": "Isozymes of glyceraldehyde-3-phosphate dehydrogenase in man and other mammals.", "journal": "Ann Hum Genet", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/183598/" }, { "id": "PMID:10714828", "title": "Human glyceraldehyde 3-phosphate dehydrogenase-2 gene is expressed specifically in spermatogenic cells.", "journal": "J Androl", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10714828/" }, { "id": "PMID:3170585", "title": "Isolation and complete sequence of a functional human glyceraldehyde-3-phosphate dehydrogenase gene", "journal": "J Biol Chem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3170585/" } ], "x": 1530, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70486", "label": "PGK complexes (PGK1,2) phosphorylate 3PG to form 1,3BPG", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14802" ], "source_url": "https://reactome.org/content/detail/R-HSA-70486", "summary": "PGK complexes (PGK1,2) phosphorylate 3PG to form 1,3BPG is a Reactome release 97 curated reaction event. The source record connects ATP, 3PG to ADP, 1,3BPG in cytosol and cross-references RHEA:14802.", "equation": "ATP + 3PG → ADP + 1,3BPG", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29728", "reactome_id": "R-ALL-29728", "label": "3PG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58272", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29800", "reactome_id": "R-ALL-29800", "label": "1,3BPG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57604", "formula": "C3H4O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phosphoglycerate kinase activity of PGK complexes [cytosol]", "activity_id": "70485", "ec_numbers": [ "2.7.2.3" ], "accessions": null } ], "citations": [ { "id": "PMID:1278465", "title": "Characterization of phosphoglycerate kinase from human spermatozoa", "journal": "Fertil. Steril.", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/1278465/" }, { "id": "PMID:7391027", "title": "Complete amino acid sequence of human phosphoglycerate kinase. Cyanogen bromide peptides and complete amino acid sequence.", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/7391027/" }, { "id": "PMID:6771269", "title": "Complete amino acid sequence of human phosphoglycerate kinase. Isolation and amino acid sequence of tryptic peptides.", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6771269/" }, { "id": "PMID:5009693", "title": "Human phosphoglycerate kinase. I. Crystallization and characterization of normal enzyme", "journal": "J Biol Chem", "year": 1972, "url": "https://pubmed.ncbi.nlm.nih.gov/5009693/" } ], "x": 1808.6666666666667, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70494", "label": "Enolase dimers (ENO1,2,3) convert PEP to 2PG", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10166" ], "source_url": "https://reactome.org/content/detail/R-HSA-70494", "summary": "Enolase dimers (ENO1,2,3) convert PEP to 2PG is a Reactome release 97 curated reaction event. The source record connects PEP, H2O to 2PG in cytosol and cross-references RHEA:10166.", "equation": "PEP + H2O → 2PG", "inputs": [ { "node_id": "R-ALL-29492", "reactome_id": "R-ALL-29492", "label": "PEP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58702", "formula": "C3H2O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-30485", "reactome_id": "R-ALL-30485", "label": "2PG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58289", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phosphopyruvate hydratase activity of enolase dimer [cytosol]", "activity_id": "71439", "ec_numbers": [ "4.2.1.11" ], "accessions": null } ], "citations": [ { "id": "PMID:11506403", "title": "Beta-enolase deficiency, a new metabolic myopathy of distal glycolysis", "journal": "Ann Neurol", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11506403/" }, { "id": "PMID:3208766", "title": "Complete amino acid sequence of the neurone-specific gamma isozyme of enolase (NSE) from human brain and comparison with the non-neuronal alpha form (NNE)", "journal": "Eur J Biochem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3208766/" }, { "id": "PMID:3529090", "title": "Molecular cloning and nucleotide sequence of a full-length cDNA for human alpha enolase", "journal": "Proc Natl Acad Sci U S A", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3529090/" } ], "x": 1251.3333333333333, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70501", "label": "PC carboxylates PYR to OA", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis", "pyruvate" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:20845" ], "source_url": "https://reactome.org/content/detail/R-HSA-70501", "summary": "PC carboxylates PYR to OA is a Reactome release 97 curated reaction event. The source record connects ATP, PYR, HCO3- to ADP, Pi, OA in mitochondrial matrix and cross-references RHEA:20845.", "equation": "ATP + PYR + HCO3- → ADP + Pi + OA + H+", "inputs": [ { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113557", "reactome_id": "R-ALL-113557", "label": "PYR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29896", "reactome_id": "R-ALL-29896", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113587", "reactome_id": "R-ALL-113587", "label": "OA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "pyruvate carboxylase activity of PC tetramer [mitochondrial matrix]", "activity_id": "70500", "ec_numbers": [ "6.4.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:12437512", "title": "Expression and characterization of a human pyruvate carboxylase variant by retroviral gene transfer", "journal": "Biochem J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12437512/" }, { "id": "PMID:10229653", "title": "Structure, function and regulation of pyruvate carboxylase", "journal": "Biochem J", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10229653/" }, { "id": "PMID:18613815", "title": "Structure, mechanism and regulation of pyruvate carboxylase", "journal": "Biochem J", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18613815/" }, { "id": "PMID:9585002", "title": "Molecular characterization of pyruvate carboxylase deficiency in two consanguineous families", "journal": "Pediatr Res", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9585002/" }, { "id": "PMID:36261450", "title": "CryoEM structural exploration of catalytically active enzyme pyruvate carboxylase", "journal": "Nat Commun", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/36261450/" }, { "id": "PMID:4826475", "title": "Purification and properties of human liver pyruvate carboxylase", "journal": "Biochem Med", "year": 1974, "url": "https://pubmed.ncbi.nlm.nih.gov/4826475/" } ], "x": 1251.3333333333333, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70510", "label": "LDH tetramer oxidises LACT to PYR", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23445" ], "source_url": "https://reactome.org/content/detail/R-HSA-70510", "summary": "LDH tetramer oxidises LACT to PYR is a Reactome release 97 curated reaction event. The source record connects LACT, NAD+ to PYR, NADH, H+ in cytosol and cross-references RHEA:23445.", "equation": "LACT + NAD+ → PYR + NADH + H+", "inputs": [ { "node_id": "R-ALL-29708", "reactome_id": "R-ALL-29708", "label": "LACT", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16651", "formula": "C3H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-lactate dehydrogenase (NAD+) activity of LDH tetramer [cytosol]", "activity_id": "70509", "ec_numbers": [ "1.1.1.27" ], "accessions": null } ], "citations": [ { "id": "PMID:18351441", "title": "Identification of a novel human lactate dehydrogenase gene LDHAL6A, which activates transcriptional activities of AP1(PMA)", "journal": "Mol. Biol. Rep.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/18351441/" }, { "id": "PMID:1996957", "title": "Properties of human testis-specific lactate dehydrogenase expressed from Escherichia coli", "journal": "Biochem. J.", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1996957/" }, { "id": "PMID:2440048", "title": "Epitopes of human testis-specific lactate dehydrogenase deduced from a cDNA sequence", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/2440048/" }, { "id": "PMID:11276087", "title": "Structural basis for altered activity of M- and H-isozyme forms of human lactate dehydrogenase", "journal": "Proteins", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11276087/" }, { "id": "PMID:3435492", "title": "The cDNA and protein sequences of human lactate dehydrogenase B", "journal": "Biochem J", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3435492/" }, { "id": "PMID:11377399", "title": "Selective active site inhibitors of human lactate dehydrogenases A4, B4, and C4", "journal": "Biochem Pharmacol", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11377399/" } ], "x": 5008.666666666667, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70523", "label": "PXLP-K314-GPT transaminates L-Ala to form PYR", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19454" ], "source_url": "https://reactome.org/content/detail/R-HSA-70523", "summary": "PXLP-K314-GPT transaminates L-Ala to form PYR is a Reactome release 97 curated reaction event. The source record connects L-Ala, 2OG to PYR, L-Glu in cytosol and cross-references RHEA:19454.", "equation": "L-Ala + 2OG → PYR + L-Glu", "inputs": [ { "node_id": "R-ALL-29432", "reactome_id": "R-ALL-29432", "label": "L-Ala", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57972", "formula": "C3H7NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-alanine:2-oxoglutarate transaminase activity of PXLP-K314-GPT dimer [cytosol]", "activity_id": "70522", "ec_numbers": [ "2.6.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:1931970", "title": "Complete amino acid sequence of human liver cytosolic alanine aminotransferase (GPT) determined by a combination of conventional and mass spectral methods", "journal": "Biochemistry", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1931970/" }, { "id": "PMID:9119391", "title": "Human glutamate pyruvate transaminase (GPT): localization to 8q24.3, cDNA and genomic sequences, and polymorphic sites.", "journal": "Genomics", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9119391/" }, { "id": "PMID:11863375", "title": "cDNA cloning, genomic structure, chromosomal mapping, and functional expression of a novel human alanine aminotransferase", "journal": "Genomics", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11863375/" } ], "x": 4730, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70542", "label": "glutamate + acetyl CoA =\u003e N-acetyl glutamate + CoA", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:24293" ], "source_url": "https://reactome.org/content/detail/R-HSA-70542", "summary": "glutamate + acetyl CoA =\u003e N-acetyl glutamate + CoA is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, L-Glu to CoA-SH, NAcGlu, H+ in mitochondrial matrix and cross-references RHEA:24293.", "equation": "Ac-CoA + L-Glu → CoA-SH + NAcGlu + H+", "inputs": [ { "node_id": "R-ALL-113559", "reactome_id": "R-ALL-113559", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-30471", "reactome_id": "R-ALL-30471", "label": "NAcGlu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:44337", "formula": "C7H9NO5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-glutamate N-acetyltransferase activity, acting on acetyl-CoA as donor of NAGS(19-534) dimer [mitochondrial matrix]", "activity_id": "70539", "ec_numbers": [ "2.3.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:12459178", "title": "Cloning and expression of the human N-acetylglutamate synthase gene.", "journal": "Biochem Biophys Res Commun", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12459178/" }, { "id": "PMID:15050968", "title": "Mammalian N-acetylglutamate synthase", "journal": "Mol Genet Metab", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15050968/" } ], "x": 1808.6666666666667, "y": 3160.5555555555557, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70555", "label": "2 ATP + NH4+ + HCO3- =\u003e 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:18030" ], "source_url": "https://reactome.org/content/detail/R-HSA-70555", "summary": "2 ATP + NH4+ + HCO3- =\u003e 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] is a Reactome release 97 curated reaction event. The source record connects HCO3-, NH4+, ATP to CAP, Pi, ADP in mitochondrial matrix and cross-references RHEA:18030.", "equation": "HCO3- + NH4+ + 2 ATP → CAP + Pi + 2 ADP + 2 H+", "inputs": [ { "node_id": "R-ALL-29896", "reactome_id": "R-ALL-29896", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113561", "reactome_id": "R-ALL-113561", "label": "NH4+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29676", "reactome_id": "R-ALL-29676", "label": "CAP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58228", "formula": "CH2NO5P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "carbamoyl-phosphate synthase (ammonia) activity of CPS1 [mitochondrial matrix]", "activity_id": "70552", "ec_numbers": [ "6.3.4.16" ], "accessions": null } ], "citations": [ { "id": "PMID:6249820", "title": "Human carbamylphosphate synthetase I. Stabilization, purification, and partial characterization of the enzyme from human liver.", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6249820/" }, { "id": "PMID:7028477", "title": "Human carbamoylphosphate synthetase I", "journal": "Enzyme", "year": 1981, "url": "https://pubmed.ncbi.nlm.nih.gov/7028477/" }, { "id": "PMID:19754428", "title": "Structural insight on the control of urea synthesis: identification of the binding site for N-acetyl-L-glutamate, the essential allosteric activator of mitochondrial carbamoyl phosphate synthetase", "journal": "Biochem J", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19754428/" }, { "id": "PMID:23649895", "title": "Molecular characterization of carbamoyl-phosphate synthetase (CPS1) deficiency using human recombinant CPS1 as a key tool", "journal": "Hum Mutat", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23649895/" }, { "id": "PMID:20800523", "title": "Genetic, structural and biochemical basis of carbamoyl phosphate synthetase 1 deficiency", "journal": "Mol Genet Metab", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20800523/" }, { "id": "PMID:33317798", "title": "CPS1: Looking at an ancient enzyme in a modern light", "journal": "Mol Genet Metab", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/33317798/" } ], "x": 1251.3333333333333, "y": 3160.5555555555557, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70560", "label": "carbamoyl phosphate + ornithine =\u003e citrulline + orthophosphate", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:19514" ], "source_url": "https://reactome.org/content/detail/R-HSA-70560", "summary": "carbamoyl phosphate + ornithine =\u003e citrulline + orthophosphate is a Reactome release 97 curated reaction event. The source record connects CAP, L-Orn to L-Cit, Pi, H+ in mitochondrial matrix and cross-references RHEA:19514.", "equation": "CAP + L-Orn → L-Cit + Pi + H+", "inputs": [ { "node_id": "R-ALL-29676", "reactome_id": "R-ALL-29676", "label": "CAP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58228", "formula": "CH2NO5P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113585", "reactome_id": "R-ALL-113585", "label": "L-Orn", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46911", "formula": "C5H13N2O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113567", "reactome_id": "R-ALL-113567", "label": "L-Cit", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57743", "formula": "C6H13N3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ornithine carbamoyltransferase activity of OTC trimer [mitochondrial matrix]", "activity_id": "70559", "ec_numbers": [ "2.1.3.3" ], "accessions": null } ], "citations": [ { "id": "PMID:6372096", "title": "Structure and expression of a complementary DNA for the nuclear coded precursor of human mitochondrial ornithine transcarbamylase.", "journal": "Science", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6372096/" }, { "id": "PMID:11237854", "title": "Human ornithine transcarbamylase: crystallographic insights into substrate recognition and conformational changes", "journal": "Biochem J", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11237854/" } ], "x": 1251.3333333333333, "y": 3237, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70569", "label": "arginine + H2O =\u003e ornithine + urea [ARG1]", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:20570" ], "source_url": "https://reactome.org/content/detail/R-HSA-70569", "summary": "arginine + H2O =\u003e ornithine + urea [ARG1] is a Reactome release 97 curated reaction event. The source record connects L-Arg, H2O to L-Orn, Urea in cytosol and cross-references RHEA:20570.", "equation": "L-Arg + H2O → L-Orn + Urea", "inputs": [ { "node_id": "R-ALL-29468", "reactome_id": "R-ALL-29468", "label": "L-Arg", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:32682", "formula": "C6H15N4O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29498", "reactome_id": "R-ALL-29498", "label": "L-Orn", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:46911", "formula": "C5H13N2O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29514", "reactome_id": "R-ALL-29514", "label": "Urea", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16199", "formula": "CH4N2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "arginase activity of ARG1 trimer [cytosol]", "activity_id": "70568", "ec_numbers": [ "3.5.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:16141327", "title": "Crystal structure of human arginase I at 1.29-A resolution and exploration of inhibition in the immune response", "journal": "Proc Natl Acad Sci U S A", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16141327/" }, { "id": "PMID:7649538", "title": "Molecular basis of phenotypic variation in patients with argininemia", "journal": "Hum Genet", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7649538/" } ], "x": 1808.6666666666667, "y": 3237, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70573", "label": "argininosuccinate fumarate + arginine", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24021" ], "source_url": "https://reactome.org/content/detail/R-HSA-70573", "summary": "argininosuccinate fumarate + arginine is a Reactome release 97 curated reaction event. The source record connects ARSUA to L-Arg, FUMA in cytosol and cross-references RHEA:24021.", "equation": "ARSUA → L-Arg + FUMA", "inputs": [ { "node_id": "R-ALL-34575", "reactome_id": "R-ALL-34575", "label": "ARSUA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57472", "formula": "C10H17N4O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29468", "reactome_id": "R-ALL-29468", "label": "L-Arg", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:32682", "formula": "C6H15N4O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29586", "reactome_id": "R-ALL-29586", "label": "FUMA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29806", "formula": "C4H2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "argininosuccinate lyase activity of ASL tetramer [cytosol]", "activity_id": "70572", "ec_numbers": [ "4.3.2.1" ], "accessions": null } ], "citations": [ { "id": "PMID:9256435", "title": "Human argininosuccinate lyase: a structural basis for intragenic complementation", "journal": "Proc Natl Acad Sci U S A", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9256435/" }, { "id": "PMID:2263616", "title": "Molecular analysis of human argininosuccinate lyase: mutant characterization and alternative splicing of the coding region", "journal": "Proc Natl Acad Sci U S A", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2263616/" } ], "x": 1251.3333333333333, "y": 3313.444444444445, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70577", "label": "ASS1 tetramer:NMRAL1 dimer:NADPH transforms L-Asp and L-Cit to ARSUA", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10933" ], "source_url": "https://reactome.org/content/detail/R-HSA-70577", "summary": "ASS1 tetramer:NMRAL1 dimer:NADPH transforms L-Asp and L-Cit to ARSUA is a Reactome release 97 curated reaction event. The source record connects ATP, L-Asp, L-Cit to ARSUA, AMP, PPi in cytosol and cross-references RHEA:10933.", "equation": "ATP + L-Asp + L-Cit → ARSUA + AMP + PPi + H+", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29448", "reactome_id": "R-ALL-29448", "label": "L-Asp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29968", "reactome_id": "R-ALL-29968", "label": "L-Cit", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57743", "formula": "C6H13N3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-34575", "reactome_id": "R-ALL-34575", "label": "ARSUA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57472", "formula": "C10H17N4O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "argininosuccinate synthase activity of ASS1 tetramer:NMRAL1 dimer:NADPH [cytosol]", "activity_id": "70576", "ec_numbers": [ "6.3.4.5" ], "accessions": null } ], "citations": [ { "id": "PMID:19006241", "title": "Mutations and polymorphisms in the human argininosuccinate synthetase (ASS1) gene", "journal": "Hum Mutat", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19006241/" }, { "id": "PMID:18323623", "title": "Structure of human argininosuccinate synthetase", "journal": "Acta Crystallogr D Biol Crystallogr", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18323623/" }, { "id": "PMID:518841", "title": "Isolation and characterization of argininosuccinate synthetase from human liver.", "journal": "Biochemistry", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/518841/" }, { "id": "PMID:17496144", "title": "Restructuring of the dinucleotide-binding fold in an NADP(H) sensor protein", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17496144/" }, { "id": "PMID:18263583", "title": "An NADPH sensor protein (HSCARG) down-regulates nitric oxide synthesis by association with argininosuccinate synthetase and is essential for epithelial cell viability", "journal": "J. Biol. Chem.", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18263583/" } ], "x": 1530, "y": 3160.5555555555557, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70589", "label": "alpha-ketoglutarate + NH4+ + NAD(P)H + H+ glutamate + NAD(P)+ (GLUD1,2)", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:15135", "RHEA:11614" ], "source_url": "https://reactome.org/content/detail/R-HSA-70589", "summary": "alpha-ketoglutarate + NH4+ + NAD(P)H + H+ glutamate + NAD(P)+ (GLUD1,2) is a Reactome release 97 curated reaction event. The source record connects 2OG, NH4+, NAD(P)H to L-Glu, NAD(P)+, H2O in mitochondrial matrix and cross-references RHEA:15135, RHEA:11614.", "equation": "2OG + NH4+ + NAD(P)H + H+ → L-Glu + NAD(P)+ + H2O", "inputs": [ { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113561", "reactome_id": "R-ALL-113561", "label": "NH4+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-517496", "reactome_id": "R-ALL-517496", "label": "NAD(P)H", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-517495", "reactome_id": "R-ALL-517495", "label": "NAD(P)+", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-glutamate dehydrogenase [NAD(P)+] activity of GLUD hexamer [mitochondrial matrix]", "activity_id": "70584", "ec_numbers": [ "1.4.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:11903050", "title": "Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations", "journal": "Biochem J", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11903050/" }, { "id": "PMID:429360", "title": "Partial amino acid sequence of the glutamate dehydrogenase of human liver and a revision of the sequence of the bovine enzyme", "journal": "J Biol Chem", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/429360/" }, { "id": "PMID:12054821", "title": "The structure of apo human glutamate dehydrogenase details subunit communication and allostery", "journal": "J Mol Biol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12054821/" } ], "x": 8018.666666666667, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70592", "label": "PXLP-K259-GOT1 transaminates 2-OG and L-Asp", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport", "malate-aspartate-shuttle" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:21825" ], "source_url": "https://reactome.org/content/detail/R-HSA-70592", "summary": "PXLP-K259-GOT1 transaminates 2-OG and L-Asp is a Reactome release 97 curated reaction event. The source record connects L-Asp, 2OG to OA, L-Glu in cytosol and cross-references RHEA:21825.", "equation": "L-Asp + 2OG → OA + L-Glu", "inputs": [ { "node_id": "R-ALL-29448", "reactome_id": "R-ALL-29448", "label": "L-Asp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76213", "reactome_id": "R-ALL-76213", "label": "OA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-aspartate:2-oxoglutarate transaminase activity of PXLP-K259-GOT1 dimer [cytosol]", "activity_id": "70580", "ec_numbers": [ "2.6.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:2241899", "title": "The amino acid sequence of cytosolic aspartate aminotransferase from human liver", "journal": "Biochem J", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2241899/" }, { "id": "PMID:30365304", "title": "Biochemical Characterization and Structure-Based Mutational Analysis Provide Insight into the Binding and Mechanism of Action of Novel Aspartate Aminotransferase Inhibitors", "journal": "Biochemistry", "year": 2018, "url": "https://pubmed.ncbi.nlm.nih.gov/30365304/" } ], "x": 5008.666666666667, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70600", "label": "glutamate + NAD(P)+ =\u003e alpha-ketoglutarate + NH4+ + NAD(P)H + H+ (GLUD1,2)", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:15134", "RHEA:11613" ], "source_url": "https://reactome.org/content/detail/R-HSA-70600", "summary": "glutamate + NAD(P)+ =\u003e alpha-ketoglutarate + NH4+ + NAD(P)H + H+ (GLUD1,2) is a Reactome release 97 curated reaction event. The source record connects L-Glu, NAD(P)+, H2O to 2OG, NH4+, NAD(P)H in mitochondrial matrix and cross-references RHEA:15134, RHEA:11613.", "equation": "L-Glu + NAD(P)+ + H2O → 2OG + NH4+ + NAD(P)H + H+", "inputs": [ { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-517495", "reactome_id": "R-ALL-517495", "label": "NAD(P)+", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113561", "reactome_id": "R-ALL-113561", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-517496", "reactome_id": "R-ALL-517496", "label": "NAD(P)H", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-glutamate dehydrogenase [NAD(P)+] activity of GLUD hexamer [mitochondrial matrix]", "activity_id": "70584", "ec_numbers": [ "1.4.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:11903050", "title": "Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations", "journal": "Biochem J", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11903050/" }, { "id": "PMID:429360", "title": "Partial amino acid sequence of the glutamate dehydrogenase of human liver and a revision of the sequence of the bovine enzyme", "journal": "J Biol Chem", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/429360/" }, { "id": "PMID:12054821", "title": "The structure of apo human glutamate dehydrogenase details subunit communication and allostery", "journal": "J Mol Biol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12054821/" } ], "x": 7461.333333333333, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70606", "label": "glutamate + NH4+ + ATP =\u003e glutamine + ADP + orthophosphate [GLUL]", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:16170" ], "source_url": "https://reactome.org/content/detail/R-HSA-70606", "summary": "glutamate + NH4+ + ATP =\u003e glutamine + ADP + orthophosphate [GLUL] is a Reactome release 97 curated reaction event. The source record connects ATP, L-Glu, NH4+ to ADP, L-Gln, Pi in cytosol and cross-references RHEA:16170.", "equation": "ATP + L-Glu + NH4+ → ADP + L-Gln + Pi + H+", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29472", "reactome_id": "R-ALL-29472", "label": "L-Gln", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58359", "formula": "C5H10N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glutamine synthetase activity of GLUL decamer [cytosol]", "activity_id": "70605", "ec_numbers": [ "6.3.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:16267323", "title": "Congenital glutamine deficiency with glutamine synthetase mutations", "journal": "N Engl J Med", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16267323/" }, { "id": "PMID:18005987", "title": "Crystal structures of mammalian glutamine synthetases illustrate substrate-induced conformational changes and provide opportunities for drug and herbicide design", "journal": "J Mol Biol", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18005987/" } ], "x": 7740, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70609", "label": "glutamine + H2O =\u003e glutamate + NH4+ (GLS)", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:15890" ], "source_url": "https://reactome.org/content/detail/R-HSA-70609", "summary": "glutamine + H2O =\u003e glutamate + NH4+ (GLS) is a Reactome release 97 curated reaction event. The source record connects H2O, L-Gln to L-Glu, NH4+ in mitochondrial matrix and cross-references RHEA:15890.", "equation": "H2O + L-Gln → L-Glu + NH4+", "inputs": [ { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113522", "reactome_id": "R-ALL-113522", "label": "L-Gln", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58359", "formula": "C5H10N2O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113561", "reactome_id": "R-ALL-113561", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glutaminase activity of GLS dimers [mitochondrial matrix]", "activity_id": "70608", "ec_numbers": [ "3.5.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:11015561", "title": "Cloning and analysis of unique human glutaminase isoforms generated by tissue-specific alternative splicing", "journal": "Physiol Genomics", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/11015561/" }, { "id": "PMID:10620514", "title": "Molecular cloning, sequencing and expression studies of the human breast cancer cell glutaminase", "journal": "Biochem J", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10620514/" }, { "id": "PMID:3214421", "title": "Purification of phosphate-dependent glutaminase from isolated mitochondria of Ehrlich ascites-tumour cells", "journal": "Biochem J", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3214421/" } ], "x": 8018.666666666667, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70613", "label": "GOT2 transaminates OA and L-Glu", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport", "glutamate-glutamine", "malate-aspartate-shuttle" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:21826" ], "source_url": "https://reactome.org/content/detail/R-HSA-70613", "summary": "GOT2 transaminates OA and L-Glu is a Reactome release 97 curated reaction event. The source record connects OA, L-Glu to L-Asp, 2OG in mitochondrial matrix and cross-references RHEA:21826.", "equation": "OA + L-Glu → L-Asp + 2OG", "inputs": [ { "node_id": "R-ALL-113587", "reactome_id": "R-ALL-113587", "label": "OA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113553", "reactome_id": "R-ALL-113553", "label": "L-Asp", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-aspartate:2-oxoglutarate transaminase activity of GOT2 dimer [mitochondrial matrix]", "activity_id": "70595", "ec_numbers": [ "2.6.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:4052435", "title": "The primary structure of mitochondrial aspartate aminotransferase from human heart", "journal": "Biochim Biophys Acta", "year": 1985, "url": "https://pubmed.ncbi.nlm.nih.gov/4052435/" }, { "id": "PMID:31422819", "title": "Bi-allelic GOT2 Mutations Cause a Treatable Malate-Aspartate Shuttle-Related Encephalopathy", "journal": "Am J Hum Genet", "year": 2019, "url": "https://pubmed.ncbi.nlm.nih.gov/31422819/" }, { "id": "PMID:26902786", "title": "Recombinant expression, purification and crystallographic studies of the mature form of human mitochondrial aspartate aminotransferase", "journal": "Biosci Trends", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/26902786/" } ], "x": 4451.333333333333, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70634", "label": "ornithine (cytosolic) + citrulline (mitochondrial) =\u003e ornithine (mitochondrial) + citrulline (cytosolic)", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "cytosol · mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005829", "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:70787" ], "source_url": "https://reactome.org/content/detail/R-HSA-70634", "summary": "ornithine (cytosolic) + citrulline (mitochondrial) =\u003e ornithine (mitochondrial) + citrulline (cytosolic) is a Reactome release 97 curated reaction event. The source record connects L-Cit, L-Orn, H+ to L-Cit, L-Orn, H+ in cytosol · mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:70787.", "equation": "L-Cit + L-Orn + H+ → L-Cit + L-Orn + H+", "inputs": [ { "node_id": "R-ALL-113567", "reactome_id": "R-ALL-113567", "label": "L-Cit", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57743", "formula": "C6H13N3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29498", "reactome_id": "R-ALL-29498", "label": "L-Orn", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46911", "formula": "C5H13N2O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29968", "reactome_id": "R-ALL-29968", "label": "L-Cit", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57743", "formula": "C6H13N3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113585", "reactome_id": "R-ALL-113585", "label": "L-Orn", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:46911", "formula": "C5H13N2O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-ornithine transmembrane transporter activity of Mitochondrial ornithine transporters [mitochondrial inner membrane]", "activity_id": "70633", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10369256", "title": "Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter.", "journal": "Nat Genet", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10369256/" }, { "id": "PMID:12807890", "title": "The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12807890/" }, { "id": "PMID:12948741", "title": "Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder", "journal": "Mol Genet Metab", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12948741/" } ], "x": 1530, "y": 3313.444444444445, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70654", "label": "ornithine + alpha-ketoglutarate glutamate + L-glutamate gamma-semialdehyde [OAT]", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:25161" ], "source_url": "https://reactome.org/content/detail/R-HSA-70654", "summary": "ornithine + alpha-ketoglutarate glutamate + L-glutamate gamma-semialdehyde [OAT] is a Reactome release 97 curated reaction event. The source record connects L-Orn, 2OG to L-Glu, L-Glu5S in mitochondrial matrix and cross-references RHEA:25161.", "equation": "L-Orn + 2OG → L-Glu + L-Glu5S", "inputs": [ { "node_id": "R-ALL-113585", "reactome_id": "R-ALL-113585", "label": "L-Orn", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46911", "formula": "C5H13N2O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31339", "reactome_id": "R-ALL-31339", "label": "L-Glu5S", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58066", "formula": "C5H9NO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-ornithine transaminase activity of OAT hexamer [mitochondrial matrix]", "activity_id": "70653", "ec_numbers": [ "2.6.1.13" ], "accessions": null } ], "citations": [ { "id": "PMID:1737786", "title": "Ornithine delta-aminotransferase mutations in gyrate atrophy. Allelic heterogeneity and functional consequences.", "journal": "J Biol Chem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1737786/" }, { "id": "PMID:6819292", "title": "Crystallization and properties of human liver ornithine aminotransferase", "journal": "J Biochem", "year": 1982, "url": "https://pubmed.ncbi.nlm.nih.gov/6819292/" }, { "id": "PMID:9514741", "title": "Crystal structure of human recombinant ornithine aminotransferase.", "journal": "J Mol Biol", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9514741/" } ], "x": 7461.333333333333, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70664", "label": "PYCR1 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:14107" ], "source_url": "https://reactome.org/content/detail/R-HSA-70664", "summary": "PYCR1 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro is a Reactome release 97 curated reaction event. The source record connects 1PYR-5COOH, H+, NADH to L-Pro, NAD+ in mitochondrial matrix and cross-references RHEA:14107.", "equation": "1PYR-5COOH + 2 H+ + NADH → L-Pro + NAD+", "inputs": [ { "node_id": "R-ALL-113590", "reactome_id": "R-ALL-113590", "label": "1PYR-5COOH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17388", "formula": "C5H6NO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-379717", "reactome_id": "R-ALL-379717", "label": "L-Pro", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:60039", "formula": "C5H9NO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "pyrroline-5-carboxylate reductase activity of PYCR1 decamer [mitochondrial matrix]", "activity_id": "70663", "ec_numbers": [ "1.5.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:23024808", "title": "Functional specialization in proline biosynthesis of melanoma", "journal": "PLoS ONE", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/23024808/" }, { "id": "PMID:16730026", "title": "Crystal structure of human pyrroline-5-carboxylate reductase", "journal": "J Mol Biol", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16730026/" }, { "id": "PMID:19648921", "title": "Mutations in PYCR1 cause cutis laxa with progeroid features", "journal": "Nat Genet", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19648921/" } ], "x": 7740, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70723", "label": "leu, ile, or val + alpha-ketoglutarate a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT1]", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18322", "RHEA:24802", "RHEA:24814" ], "source_url": "https://reactome.org/content/detail/R-HSA-70723", "summary": "leu, ile, or val + alpha-ketoglutarate a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT1] is a Reactome release 97 curated reaction event. The source record connects Leu, Ile, Val, 2OG to KIC,KMVA,KIV, L-Glu in cytosol and cross-references RHEA:18322, RHEA:24802, RHEA:24814.", "equation": "Leu, Ile, Val + 2OG → KIC,KMVA,KIV + L-Glu", "inputs": [ { "node_id": "R-ALL-508182", "reactome_id": "R-ALL-508182", "label": "Leu, Ile, Val", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-508187", "reactome_id": "R-ALL-508187", "label": "KIC,KMVA,KIV", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "branched-chain-amino-acid:2-oxoglutarate transaminase activity of BCAT1 dimer [cytosol]", "activity_id": "70700", "ec_numbers": [ "2.6.1.42" ], "accessions": null } ], "citations": [ { "id": "PMID:16141215", "title": "Structural determinants for branched-chain aminotransferase isozyme-specific inhibition by the anticonvulsant drug gabapentin", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16141215/" } ], "x": 6800, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70724", "label": "leu, ile, or val + alpha-ketoglutarate a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2]", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:24814", "RHEA:18322", "RHEA:24802" ], "source_url": "https://reactome.org/content/detail/R-HSA-70724", "summary": "leu, ile, or val + alpha-ketoglutarate a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] is a Reactome release 97 curated reaction event. The source record connects Leu, Ile, Val, 2OG to KIC, KMVA, KIV, L-Glu in mitochondrial matrix and cross-references RHEA:24814, RHEA:18322, RHEA:24802.", "equation": "Leu, Ile, Val + 2OG → KIC, KMVA, KIV + L-Glu", "inputs": [ { "node_id": "R-ALL-508190", "reactome_id": "R-ALL-508190", "label": "Leu, Ile, Val", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-508181", "reactome_id": "R-ALL-508181", "label": "KIC, KMVA, KIV", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "branched-chain-amino-acid:2-oxoglutarate transaminase activity of BCAT2 dimer [mitochondrial matrix]", "activity_id": "70711", "ec_numbers": [ "2.6.1.42" ], "accessions": null } ], "citations": [ { "id": "PMID:9165094", "title": "Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm)", "journal": "Biochim Biophys Acta", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9165094/" }, { "id": "PMID:11264579", "title": "The structure of human mitochondrial branched-chain aminotransferase.", "journal": "Acta Crystallogr D Biol Crystallogr", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11264579/" }, { "id": "PMID:12269802", "title": "Crystal structures of human mitochondrial branched chain aminotransferase reaction intermediates: ketimine and pyridoxamine phosphate forms", "journal": "Biochemistry", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12269802/" } ], "x": 7078.666666666667, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70773", "label": "beta-methylcrotonyl-CoA + ATP + HCO3- beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:13590" ], "source_url": "https://reactome.org/content/detail/R-HSA-70773", "summary": "beta-methylcrotonyl-CoA + ATP + HCO3- beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) is a Reactome release 97 curated reaction event. The source record connects bMC-CoA, ATP, HCO3- to bMG-CoA, ADP, Pi in mitochondrial matrix and cross-references RHEA:13590.", "equation": "bMC-CoA + ATP + HCO3- → bMG-CoA + ADP + Pi + H+", "inputs": [ { "node_id": "R-ALL-70744", "reactome_id": "R-ALL-70744", "label": "bMC-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57344", "formula": "C26H38N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29896", "reactome_id": "R-ALL-29896", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-70772", "reactome_id": "R-ALL-70772", "label": "bMG-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57346", "formula": "C27H37N7O19P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "methylcrotonoyl-CoA carboxylase activity of 6x(Btn-MCCC1:MCCC2) [mitochondrial matrix]", "activity_id": "70771", "ec_numbers": [ "6.4.1.4" ], "accessions": null } ], "citations": [ { "id": "PMID:11181649", "title": "The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency.", "journal": "J Clin Invest", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11181649/" }, { "id": "PMID:7356336", "title": "Subcellular localization of 3-methylcrotonyl-coenzyme A carboxylase in bovine kidney", "journal": "Arch Biochem Biophys", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/7356336/" }, { "id": "PMID:11406611", "title": "Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency.", "journal": "Hum Mol Genet", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11406611/" }, { "id": "PMID:16023992", "title": "Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase", "journal": "Biochem Biophys Res Commun", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16023992/" } ], "x": 6800, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70837", "label": "alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ alpha-methylacetoacetyl-CoA + NADH + H+", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:13282" ], "source_url": "https://reactome.org/content/detail/R-HSA-70837", "summary": "alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ alpha-methylacetoacetyl-CoA + NADH + H+ is a Reactome release 97 curated reaction event. The source record connects aMbHBUT-CoA, NAD+ to 2MACA-CoA, NADH, H+ in mitochondrial matrix and cross-references RHEA:13282.", "equation": "aMbHBUT-CoA + NAD+ → 2MACA-CoA + NADH + H+", "inputs": [ { "node_id": "R-ALL-70829", "reactome_id": "R-ALL-70829", "label": "aMbHBUT-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57312", "formula": "C26H40N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-70836", "reactome_id": "R-ALL-70836", "label": "2MACA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57335", "formula": "C26H38N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of HSD17B10 tetramer [mitochondrial matrix]", "activity_id": "70835", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:15342248", "title": "Crystal structure of human ABAD/HSD10 with a bound inhibitor: implications for design of Alzheimer's disease therapeutics", "journal": "J Mol Biol", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15342248/" }, { "id": "PMID:12696021", "title": "2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene.", "journal": "Am J Hum Genet", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12696021/" } ], "x": 6800, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70881", "label": "beta-hydroxyisobutyryl-CoA + H2O =\u003e beta-hydroxyisobutyrate + CoA", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31252" ], "source_url": "https://reactome.org/content/detail/R-HSA-70881", "summary": "beta-hydroxyisobutyryl-CoA + H2O =\u003e beta-hydroxyisobutyrate + CoA is a Reactome release 97 curated reaction event. The source record connects bHIB-CoA, H2O to bHIBA, CoA-SH, H+ in mitochondrial matrix and cross-references RHEA:31252.", "equation": "bHIB-CoA + H2O → bHIBA + CoA-SH + H+", "inputs": [ { "node_id": "R-ALL-71847", "reactome_id": "R-ALL-71847", "label": "bHIB-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62611", "formula": "C25H38N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-573404", "reactome_id": "R-ALL-573404", "label": "bHIBA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62638", "formula": "C4H7O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "3-hydroxyisobutyryl-CoA hydrolase activity of HIBCH [mitochondrial matrix]", "activity_id": "70880", "ec_numbers": [ "3.1.2.4" ], "accessions": null } ], "citations": [ { "id": "PMID:8824301", "title": "Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase.", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8824301/" } ], "x": 7078.666666666667, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70893", "label": "methylmalonate semialdehyde + NAD+ + CoA + H2O =\u003e propionyl-CoA + HCO3- + NADH + H+", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:76628" ], "source_url": "https://reactome.org/content/detail/R-HSA-70893", "summary": "methylmalonate semialdehyde + NAD+ + CoA + H2O =\u003e propionyl-CoA + HCO3- + NADH + H+ is a Reactome release 97 curated reaction event. The source record connects 2M3OPROA, NAD+, CoA-SH to PROP-CoA, NADH, H+ in mitochondrial matrix and cross-references RHEA:76628.", "equation": "2M3OPROA + NAD+ + CoA-SH + H2O → PROP-CoA + NADH + H+ + HCO3-", "inputs": [ { "node_id": "R-ALL-30004", "reactome_id": "R-ALL-30004", "label": "2M3OPROA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62413", "formula": "C4H5O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-70843", "reactome_id": "R-ALL-70843", "label": "PROP-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57392", "formula": "C24H36N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29896", "reactome_id": "R-ALL-29896", "label": "HCO3-", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "methylmalonate-semialdehyde dehydrogenase (acylating, NAD) activity of ALDH6A1 [mitochondrial matrix]", "activity_id": "70892", "ec_numbers": [ "1.2.1.27" ], "accessions": null } ], "citations": [ { "id": "PMID:10947204", "title": "Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency", "journal": "J Inherit Metab Dis", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10947204/" }, { "id": "PMID:1527093", "title": "CoA-dependent methylmalonate-semialdehyde dehydrogenase, a unique member of the aldehyde dehydrogenase superfamily. cDNA cloning, evolutionary relationships, and tissue distribution", "journal": "J Biol Chem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1527093/" } ], "x": 7078.666666666667, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70967", "label": "IDH3 complex decarboxylates isocitrate", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:23633" ], "source_url": "https://reactome.org/content/detail/R-HSA-70967", "summary": "IDH3 complex decarboxylates isocitrate is a Reactome release 97 curated reaction event. The source record connects ISCIT, NAD+ to 2OG, CO2, NADH in mitochondrial matrix and cross-references RHEA:23633.", "equation": "ISCIT + NAD+ → 2OG + CO2 + NADH", "inputs": [ { "node_id": "R-ALL-29938", "reactome_id": "R-ALL-29938", "label": "ISCIT", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15562", "formula": "C6H5O7", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "isocitrate dehydrogenase (NAD+) activity of IDH3 octamer [mitochondrial matrix]", "activity_id": "70966", "ec_numbers": [ "1.1.1.41" ], "accessions": null } ], "citations": [ { "id": "PMID:17432878", "title": "Role of alpha-Asp181, beta-Asp192, and gamma-Asp190 in the distinctive subunits of human NAD-specific isocitrate dehydrogenase", "journal": "Biochemistry", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17432878/" }, { "id": "PMID:20435888", "title": "Each conserved active site tyr in the three subunits of human isocitrate dehydrogenase has a different function", "journal": "J Biol Chem", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20435888/" }, { "id": "PMID:14555658", "title": "Evaluation by mutagenesis of the importance of 3 arginines in alpha, beta, and gamma subunits of human NAD-dependent isocitrate dehydrogenase", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14555658/" }, { "id": "PMID:16737955", "title": "Identification of Mn2+-binding aspartates from alpha, beta, and gamma subunits of human NAD-dependent isocitrate dehydrogenase", "journal": "J Biol Chem", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16737955/" }, { "id": "PMID:18806796", "title": "Insights from retinitis pigmentosa into the roles of isocitrate dehydrogenases in the Krebs cycle", "journal": "Nat Genet", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18806796/" }, { "id": "PMID:4336621", "title": "Diphosphopyridine nucleotide dependent isocitrate dehydrogenase from pig heart. Charactgerization of the active substrate and modes of regulation", "journal": "Biochemistry", "year": 1972, "url": "https://pubmed.ncbi.nlm.nih.gov/4336621/" } ], "x": 5391.333333333333, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70971", "label": "ACO2 isomerizes citrate", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:10337", "RHEA:10336" ], "source_url": "https://reactome.org/content/detail/R-HSA-70971", "summary": "ACO2 isomerizes citrate is a Reactome release 97 curated reaction event. The source record connects CIT to ISCIT in mitochondrial matrix and cross-references RHEA:10337, RHEA:10336.", "equation": "CIT → ISCIT", "inputs": [ { "node_id": "R-ALL-29654", "reactome_id": "R-ALL-29654", "label": "CIT", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16947", "formula": "C6H5O7", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29938", "reactome_id": "R-ALL-29938", "label": "ISCIT", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15562", "formula": "C6H5O7", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "aconitate hydratase activity of ACO2:4Fe-4S [mitochondrial matrix]", "activity_id": "70969", "ec_numbers": [ "4.2.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:1946331", "title": "Purification and partial amino acid sequence of human aconitase", "journal": "Protein Seq Data Anal", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1946331/" }, { "id": "PMID:1052766", "title": "Aconitase polymorphism in man", "journal": "Ann Hum Genet", "year": 1975, "url": "https://pubmed.ncbi.nlm.nih.gov/1052766/" }, { "id": "PMID:34354088", "title": "ACO2 clinicobiological dataset with extensive phenotype ontology annotation", "journal": "Sci Data", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/34354088/" } ], "x": 5391.333333333333, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70975", "label": "CS acetylates OA to citrate", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:16846" ], "source_url": "https://reactome.org/content/detail/R-HSA-70975", "summary": "CS acetylates OA to citrate is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, H2O, OA to CIT, CoA-SH, H+ in mitochondrial matrix and cross-references RHEA:16846.", "equation": "Ac-CoA + H2O + OA → CIT + CoA-SH + H+", "inputs": [ { "node_id": "R-ALL-113559", "reactome_id": "R-ALL-113559", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113587", "reactome_id": "R-ALL-113587", "label": "OA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29654", "reactome_id": "R-ALL-29654", "label": "CIT", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16947", "formula": "C6H5O7", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "citrate synthase activity of N6,N6,N6-trimethyl-K395-CS dimer [mitochondrial matrix]", "activity_id": "70974", "ec_numbers": [ "2.3.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:6824331", "title": "Complexes between mitochondrial enzymes and either citrate synthase or glutamate dehydrogenase", "journal": "Arch Biochem Biophys", "year": 1983, "url": "https://pubmed.ncbi.nlm.nih.gov/6824331/" }, { "id": "PMID:9809442", "title": "Cloning and molecular analysis of the human citrate synthase gene", "journal": "Genome", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9809442/" }, { "id": "PMID:28887308", "title": "Uncovering human METTL12 as a mitochondrial methyltransferase that modulates citrate synthase activity through metabolite-sensitive lysine methylation", "journal": "J Biol Chem", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28887308/" }, { "id": "PMID:9792662", "title": "Interaction between citrate synthase and malate dehydrogenase. Substrate channeling of oxaloacetate", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9792662/" }, { "id": "PMID:34548590", "title": "Association of the malate dehydrogenase-citrate synthase metabolon is modulated by intermediates of the Krebs tricarboxylic acid cycle", "journal": "Sci Rep", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/34548590/" }, { "id": "PMID:25537779", "title": "Krebs cycle metabolon: structural evidence of substrate channeling revealed by cross-linking and mass spectrometry", "journal": "Angew Chem Int Ed Engl", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25537779/" } ], "x": 5948.666666666667, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70979", "label": "MDH2 dimer dehydrogenates MAL", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport", "malate-aspartate-shuttle", "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:21433" ], "source_url": "https://reactome.org/content/detail/R-HSA-70979", "summary": "MDH2 dimer dehydrogenates MAL is a Reactome release 97 curated reaction event. The source record connects MAL, NAD+ to OA, NADH, H+ in mitochondrial matrix and cross-references RHEA:21433.", "equation": "MAL + NAD+ → OA + NADH + H+", "inputs": [ { "node_id": "R-ALL-113544", "reactome_id": "R-ALL-113544", "label": "MAL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113587", "reactome_id": "R-ALL-113587", "label": "OA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-malate dehydrogenase (NAD+) activity of MDH2 dimer [mitochondrial matrix]", "activity_id": "70978", "ec_numbers": [ "1.1.1.37" ], "accessions": null } ], "citations": [ { "id": "PMID:16740313", "title": "An NADH-tetrazolium-coupled sensitive assay for malate dehydrogenase in mitochondria and crude tissue homogenates", "journal": "J Biochem Biophys Methods", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16740313/" }, { "id": "PMID:9792106", "title": "Aggregation states of mitochondrial malate dehydrogenase", "journal": "Protein Sci", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9792106/" }, { "id": "PMID:36139014", "title": "Structural Comparison of hMDH2 Complexed with Natural Substrates and Cofactors: The Importance of Phosphate Binding for Active Conformation and Catalysis", "journal": "Biomolecules", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/36139014/" }, { "id": "PMID:36420423", "title": "Malate dehydrogenase 2 deficiency is an emerging cause of pediatric epileptic encephalopathy with a recognizable biochemical signature", "journal": "Mol Genet Metab Rep", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/36420423/" }, { "id": "PMID:35366151", "title": "Palmitoylation of MDH2 by ZDHHC18 activates mitochondrial respiration and accelerates ovarian cancer growth", "journal": "Sci China Life Sci", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/35366151/" } ], "x": 5670, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70982", "label": "FH tetramer hydrates fumarate to L-malate", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:12462", "RHEA:12460" ], "source_url": "https://reactome.org/content/detail/R-HSA-70982", "summary": "FH tetramer hydrates fumarate to L-malate is a Reactome release 97 curated reaction event. The source record connects FUMA, H2O to MAL in mitochondrial matrix and cross-references RHEA:12462, RHEA:12460.", "equation": "FUMA + H2O → MAL", "inputs": [ { "node_id": "R-ALL-113588", "reactome_id": "R-ALL-113588", "label": "FUMA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29806", "formula": "C4H2O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113544", "reactome_id": "R-ALL-113544", "label": "MAL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "fumarate hydratase activity of FH tetramer [mitochondrial matrix]", "activity_id": "70981", "ec_numbers": [ "4.2.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:8200987", "title": "Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency", "journal": "J Clin Invest", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8200987/" }, { "id": "PMID:30761759", "title": "Structural, biochemical and biophysical characterization of recombinant human fumarate hydratase", "journal": "FEBS J", "year": 2019, "url": "https://pubmed.ncbi.nlm.nih.gov/30761759/" }, { "id": "PMID:37512586", "title": "The Pleiotropic Effects of Fumarate: From Mitochondrial Respiration to Epigenetic Rewiring and DNA Repair Mechanisms", "journal": "Metabolites", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/37512586/" }, { "id": "PMID:33058874", "title": "Post-translational Modifications of Fumarase Regulate its Enzyme Activity and Function in Respiration and the DNA Damage Response", "journal": "J Mol Biol", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/33058874/" } ], "x": 5948.666666666667, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70994", "label": "SDH complex dehydrogenates succinate", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix · mitochondrial inner membrane", "compartment_ids": [ "GO:0005759", "GO:0005743" ], "rhea_ids": [ "RHEA:13714" ], "source_url": "https://reactome.org/content/detail/R-HSA-70994", "summary": "SDH complex dehydrogenates succinate is a Reactome release 97 curated reaction event. The source record connects SUCCA, CoQ to FUMA, CoQH2 in mitochondrial matrix · mitochondrial inner membrane and cross-references RHEA:13714.", "equation": "SUCCA + CoQ → FUMA + CoQH2", "inputs": [ { "node_id": "R-ALL-113536", "reactome_id": "R-ALL-113536", "label": "SUCCA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30031", "formula": "C4H4O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73663", "reactome_id": "R-ALL-73663", "label": "CoQ", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:46245", "formula": "C59H90O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113588", "reactome_id": "R-ALL-113588", "label": "FUMA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29806", "formula": "C4H2O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2162198", "reactome_id": "R-ALL-2162198", "label": "CoQH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:64183", "formula": "C59H92O4", "compartment": "mitochondrial inner membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "succinate dehydrogenase (quinone) activity of SDH complex [mitochondrial inner membrane]", "activity_id": "70993", "ec_numbers": [ "1.3.5.1" ], "accessions": null } ], "citations": [ { "id": "PMID:16143825", "title": "Succinate dehydrogenase deficiency in human", "journal": "Cell Mol Life Sci", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16143825/" }, { "id": "PMID:14946179", "title": "The inhibitory effects of itaconic acid in vitro and in vivo", "journal": "J Biol Chem", "year": 1952, "url": "https://pubmed.ncbi.nlm.nih.gov/14946179/" }, { "id": "PMID:14249115", "title": "STUDIES ON SUCCINATE DEHYDROGENASE. I. SPECTRAL PROPERTIES OF THE PURIFIED ENZYME AND FORMATION OF ENZYME-COMPETITIVE INHIBITOR COMPLEXES", "journal": "Biochim Biophys Acta", "year": 1964, "url": "https://pubmed.ncbi.nlm.nih.gov/14249115/" }, { "id": "PMID:27189937", "title": "Immunoresponsive Gene 1 and Itaconate Inhibit Succinate Dehydrogenase to Modulate Intracellular Succinate Levels", "journal": "J Biol Chem", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27189937/" }, { "id": "PMID:27374498", "title": "Itaconate Links Inhibition of Succinate Dehydrogenase with Macrophage Metabolic Remodeling and Regulation of Inflammation", "journal": "Cell Metab", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27374498/" }, { "id": "PMID:15652751", "title": "Succinate links TCA cycle dysfunction to oncogenesis by inhibiting HIF-alpha prolyl hydroxylase", "journal": "Cancer Cell", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15652751/" } ], "x": 5670, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-70997", "label": "SUCLG1/A2 cleaves succinyl-CoA", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:17663", "RHEA:17662" ], "source_url": "https://reactome.org/content/detail/R-HSA-70997", "summary": "SUCLG1/A2 cleaves succinyl-CoA is a Reactome release 97 curated reaction event. The source record connects ADP, Pi, SUCC-CoA to ATP, SUCCA, CoA-SH in mitochondrial matrix and cross-references RHEA:17663, RHEA:17662.", "equation": "ADP + Pi + SUCC-CoA → ATP + SUCCA + CoA-SH", "inputs": [ { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70958", "reactome_id": "R-ALL-70958", "label": "SUCC-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57292", "formula": "C25H35N7O19P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113536", "reactome_id": "R-ALL-113536", "label": "SUCCA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30031", "formula": "C4H4O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "succinate-CoA ligase (ADP-forming) activity of SUCLA2:SUCLG1 [mitochondrial matrix]", "activity_id": "70996", "ec_numbers": [ "6.2.1.5" ], "accessions": null } ], "citations": [ { "id": "PMID:15877282", "title": "Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion", "journal": "Am J Hum Genet", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15877282/" }, { "id": "PMID:10727444", "title": "Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia", "journal": "J Clin Invest", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10727444/" }, { "id": "PMID:9765291", "title": "Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9765291/" }, { "id": "PMID:9765290", "title": "Characterization of the ATP- and GTP-specific succinyl-CoA synthetases in pigeon. The enzymes incorporate the same alpha-subunit.", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9765290/" }, { "id": "PMID:15234968", "title": "Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15234968/" }, { "id": "PMID:13181903", "title": "Guanosine triphosphate, the primary product of phosphorylation coupled to the breakdown of succinyl coenzyme A", "journal": "Biochim Biophys Acta", "year": 1954, "url": "https://pubmed.ncbi.nlm.nih.gov/13181903/" } ], "x": 5391.333333333333, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71118", "label": "PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:20274" ], "source_url": "https://reactome.org/content/detail/R-HSA-71118", "summary": "PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr is a Reactome release 97 curated reaction event. The source record connects L-Phe, BH4, O2 to L-Tyr, 4aOH-BH4 in cytosol and cross-references RHEA:20274.", "equation": "L-Phe + BH4 + O2 → L-Tyr + 4aOH-BH4", "inputs": [ { "node_id": "R-ALL-29502", "reactome_id": "R-ALL-29502", "label": "L-Phe", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58095", "formula": "C9H11NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29870", "reactome_id": "R-ALL-29870", "label": "BH4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59560", "formula": "C9H15N5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29506", "reactome_id": "R-ALL-29506", "label": "L-Tyr", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58315", "formula": "C9H11NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-71112", "reactome_id": "R-ALL-71112", "label": "4aOH-BH4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15642", "formula": "C9H15N5O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phenylalanine 4-monooxygenase activity of PAH:Fe2+ tetramer [cytosol]", "activity_id": "71074", "ec_numbers": [ "1.14.16.1" ], "accessions": null } ], "citations": [ { "id": "PMID:12126628", "title": "Crystal structure of the ternary complex of the catalytic domain of human phenylalanine hydroxylase with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine, and its implications for the mechanism of catalysis and substrate activation", "journal": "J Mol Biol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12126628/" }, { "id": "PMID:9642259", "title": "Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria.", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9642259/" }, { "id": "PMID:8889590", "title": "Phenylalanine hydroxylase deficiency in a population in Germany: mutational profile and nine novel mutations", "journal": "Hum Mutat", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8889590/" } ], "x": 6800, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71130", "label": "q-dihydrobiopterin + NADH + H+ =\u003e tetrahydrobiopterin + NAD+", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:32253" ], "source_url": "https://reactome.org/content/detail/R-HSA-71130", "summary": "q-dihydrobiopterin + NADH + H+ =\u003e tetrahydrobiopterin + NAD+ is a Reactome release 97 curated reaction event. The source record connects qDHB, NADH, H+ to BH4, NAD+ in cytosol and cross-references RHEA:32253.", "equation": "qDHB + NADH + H+ → BH4 + NAD+", "inputs": [ { "node_id": "R-ALL-71129", "reactome_id": "R-ALL-71129", "label": "qDHB", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43120", "formula": "C9H13N5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29870", "reactome_id": "R-ALL-29870", "label": "BH4", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59560", "formula": "C9H15N5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "6,7-dihydropteridine reductase activity of QDPR dimer [cytosol]", "activity_id": "71128", "ec_numbers": [ "1.5.1.34" ], "accessions": null } ], "citations": [ { "id": "PMID:3033643", "title": "Structure and expression of human dihydropteridine reductase.", "journal": "Proc Natl Acad Sci U S A", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3033643/" }, { "id": "PMID:8262916", "title": "The crystallographic structure of a human dihydropteridine reductase NADH binary complex expressed in Escherichia coli by a cDNA constructed from its rat homologue", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8262916/" } ], "x": 7078.666666666667, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71146", "label": "4a-hydroxytetrahydrobiopterin =\u003e q-dihydrobiopterin + H2O", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11921" ], "source_url": "https://reactome.org/content/detail/R-HSA-71146", "summary": "4a-hydroxytetrahydrobiopterin =\u003e q-dihydrobiopterin + H2O is a Reactome release 97 curated reaction event. The source record connects 4aOH-BH4 to qDHB, H2O in cytosol and cross-references RHEA:11921.", "equation": "4aOH-BH4 → qDHB + H2O", "inputs": [ { "node_id": "R-ALL-71112", "reactome_id": "R-ALL-71112", "label": "4aOH-BH4", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15642", "formula": "C9H15N5O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-71129", "reactome_id": "R-ALL-71129", "label": "qDHB", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43120", "formula": "C9H13N5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "4-alpha-hydroxytetrahydrobiopterin dehydratase activity of PCBD1 tetramer [cytosol]", "activity_id": "71145", "ec_numbers": [ "4.2.1.96" ], "accessions": null } ], "citations": [ { "id": "PMID:8352282", "title": "Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism.", "journal": "Am J Hum Genet", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8352282/" }, { "id": "PMID:7744010", "title": "Three-dimensional structure of the bifunctional protein PCD/DCoH, a cytoplasmic enzyme interacting with transcription factor HNF1.", "journal": "EMBO J", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7744010/" }, { "id": "PMID:8444860", "title": "Phenylalanine hydroxylase-stimulating protein/pterin-4 alpha-carbinolamine dehydratase from rat and human liver. Purification, characterization, and complete amino acid sequence.", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8444860/" } ], "x": 6521.333333333333, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71155", "label": "TAT deaminates tyrosine", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15094" ], "source_url": "https://reactome.org/content/detail/R-HSA-71155", "summary": "TAT deaminates tyrosine is a Reactome release 97 curated reaction event. The source record connects L-Tyr, 2OG to HPP, L-Glu in cytosol and cross-references RHEA:15094.", "equation": "L-Tyr + 2OG → HPP + L-Glu", "inputs": [ { "node_id": "R-ALL-29506", "reactome_id": "R-ALL-29506", "label": "L-Tyr", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58315", "formula": "C9H11NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-31365", "reactome_id": "R-ALL-31365", "label": "HPP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:36242", "formula": "C9H7O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-tyrosine:2-oxoglutarate transaminase activity of TAT dimer [cytosol]", "activity_id": "71154", "ec_numbers": [ "2.6.1.5" ], "accessions": null } ], "citations": [ { "id": "PMID:21153519", "title": "Tyrosine aminotransferase: biochemical and structural properties and molecular dynamics simulations", "journal": "Protein Cell", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/21153519/" }, { "id": "PMID:16640556", "title": "The narrow substrate specificity of human tyrosine aminotransferase--the enzyme deficient in tyrosinemia type II", "journal": "FEBS J", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16640556/" }, { "id": "PMID:16602095", "title": "The genetic tyrosinemias", "journal": "Am J Med Genet C Semin Med Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16602095/" } ], "x": 7078.666666666667, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71163", "label": "HPD dioxygenates HPP", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:16190" ], "source_url": "https://reactome.org/content/detail/R-HSA-71163", "summary": "HPD dioxygenates HPP is a Reactome release 97 curated reaction event. The source record connects HPP, O2 to HGTA, CO2 in cytosol and cross-references RHEA:16190.", "equation": "HPP + O2 → HGTA + CO2", "inputs": [ { "node_id": "R-ALL-31365", "reactome_id": "R-ALL-31365", "label": "HPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36242", "formula": "C9H7O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-30337", "reactome_id": "R-ALL-30337", "label": "HGTA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16169", "formula": "C8H7O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "4-hydroxyphenylpyruvate dioxygenase activity of HPD:AscH-:Fe2+ dimer [cytosol]", "activity_id": "71162", "ec_numbers": [ "1.13.11.27" ], "accessions": null } ], "citations": [ { "id": "PMID:10942115", "title": "Mutations in the 4-hydroxyphenylpyruvate dioxygenase gene (HPD) in patients with tyrosinemia type III", "journal": "Hum Genet", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10942115/" }, { "id": "PMID:5487549", "title": "The mechanism of enzymic formation of homogentisate from p-hydroxyphenylpyruvate", "journal": "J. Am. Chem. Soc.", "year": 1970, "url": "https://pubmed.ncbi.nlm.nih.gov/5487549/" }, { "id": "PMID:16602095", "title": "The genetic tyrosinemias", "journal": "Am J Med Genet C Semin Med Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16602095/" }, { "id": "PMID:37794595", "title": "Structure-based discovery of pyrazole-benzothiadiazole hybrid as human HPPD inhibitors", "journal": "Structure", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/37794595/" }, { "id": "PMID:32750420", "title": "Identification of key residues determining the binding specificity of human 4-hydroxyphenylpyruvate dioxygenase", "journal": "Eur J Pharm Sci", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/32750420/" }, { "id": "PMID:34047349", "title": "Functional role of residues involved in substrate binding of human 4-hydroxyphenylpyruvate dioxygenase", "journal": "Biochem J", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/34047349/" } ], "x": 6521.333333333333, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71164", "label": "HGD dioxygenates homogentisate", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15450" ], "source_url": "https://reactome.org/content/detail/R-HSA-71164", "summary": "HGD dioxygenates homogentisate is a Reactome release 97 curated reaction event. The source record connects HGTA, O2 to 4MAA, H+ in cytosol and cross-references RHEA:15450.", "equation": "HGTA + O2 → 4MAA + H+", "inputs": [ { "node_id": "R-ALL-30337", "reactome_id": "R-ALL-30337", "label": "HGTA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16169", "formula": "C8H7O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-31135", "reactome_id": "R-ALL-31135", "label": "4MAA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17105", "formula": "C8H6O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "homogentisate 1,2-dioxygenase activity of HGD hexamer [cytosol]", "activity_id": "71084", "ec_numbers": [ "1.13.11.5" ], "accessions": null } ], "citations": [ { "id": "PMID:10876237", "title": "Crystal structure of human homogentisate dioxygenase.", "journal": "Nat Struct Biol", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10876237/" }, { "id": "PMID:8782815", "title": "The molecular basis of alkaptonuria", "journal": "Nat Genet", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8782815/" }, { "id": "PMID:32158253", "title": "Alkaptonuria: Current Perspectives", "journal": "Appl Clin Genet", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/32158253/" }, { "id": "PMID:15479158", "title": "Steady-state kinetics and inhibition of anaerobically purified human homogentisate 1,2-dioxygenase", "journal": "Biochem J", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15479158/" } ], "x": 6800, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71173", "label": "GSTZ1 isomerizes 4-MAA", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14818" ], "source_url": "https://reactome.org/content/detail/R-HSA-71173", "summary": "GSTZ1 isomerizes 4-MAA is a Reactome release 97 curated reaction event. The source record connects 4MAA to 4FAA in cytosol and cross-references RHEA:14818.", "equation": "4MAA → 4FAA", "inputs": [ { "node_id": "R-ALL-31135", "reactome_id": "R-ALL-31135", "label": "4MAA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17105", "formula": "C8H6O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-31175", "reactome_id": "R-ALL-31175", "label": "4FAA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18034", "formula": "C8H6O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "maleylacetoacetate isomerase activity of GSTZ1 dimer [cytosol]", "activity_id": "71172", "ec_numbers": [ "5.2.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:11327815", "title": "Crystal structure of maleylacetoacetate isomerase/glutathione transferase zeta reveals the molecular basis for its remarkable catalytic promiscuity.", "journal": "Biochemistry", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11327815/" }, { "id": "PMID:12852784", "title": "Clarification of the role of key active site residues of glutathione transferase zeta/maleylacetoacetate isomerase by a new spectrophotometric technique", "journal": "Biochem J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12852784/" }, { "id": "PMID:24637752", "title": "Preliminary X-ray crystallographic analysis of glutathione transferase zeta 1 (GSTZ1a-1a)", "journal": "Acta Crystallogr F Struct Biol Commun", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24637752/" } ], "x": 6800, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71181", "label": "FAH cleaves 4FAA", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10245" ], "source_url": "https://reactome.org/content/detail/R-HSA-71181", "summary": "FAH cleaves 4FAA is a Reactome release 97 curated reaction event. The source record connects 4FAA, H2O to FUMA, ACA, H+ in cytosol and cross-references RHEA:10245.", "equation": "4FAA + H2O → FUMA + ACA + H+", "inputs": [ { "node_id": "R-ALL-31175", "reactome_id": "R-ALL-31175", "label": "4FAA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:18034", "formula": "C8H6O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29586", "reactome_id": "R-ALL-29586", "label": "FUMA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29806", "formula": "C4H2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29666", "reactome_id": "R-ALL-29666", "label": "ACA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13705", "formula": "C4H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "fumarylacetoacetase activity of FAH dimer [cytosol]", "activity_id": "71180", "ec_numbers": [ "3.7.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:8364576", "title": "Characterization of the human fumarylacetoacetate hydrolase gene and identification of a missense mutation abolishing enzymatic activity.", "journal": "Hum Mol Genet", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8364576/" }, { "id": "PMID:5027618", "title": "Purification and properties of a diketo acid hydrolase from beef liver", "journal": "Biochemistry", "year": 1972, "url": "https://pubmed.ncbi.nlm.nih.gov/5027618/" }, { "id": "PMID:11127930", "title": "Hereditary tyrosinaemia type I: from basics to progress in treatment", "journal": "Ann Med", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/11127930/" }, { "id": "PMID:16602095", "title": "The genetic tyrosinemias", "journal": "Am J Med Genet C Semin Med Genet", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16602095/" } ], "x": 6521.333333333333, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71188", "label": "TDO tetramer dioxygenates L-Trp to NFK", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:24537" ], "source_url": "https://reactome.org/content/detail/R-HSA-71188", "summary": "TDO tetramer dioxygenates L-Trp to NFK is a Reactome release 97 curated reaction event. The source record connects L-Trp, O2 to NFK in cytosol and cross-references RHEA:24537.", "equation": "L-Trp + O2 → NFK", "inputs": [ { "node_id": "R-ALL-29500", "reactome_id": "R-ALL-29500", "label": "L-Trp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57912", "formula": "C11H12N2O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-33179", "reactome_id": "R-ALL-33179", "label": "NFK", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58629", "formula": "C11H12N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-tryptophan 2,3-dioxygenase activity of TDO2 tetramer [cytosol]", "activity_id": "71187", "ec_numbers": [ "1.13.11.11" ], "accessions": null } ], "citations": [ { "id": "PMID:8666386", "title": "Sequence of human tryptophan 2,3-dioxygenase (TDO2): presence of a glucocorticoid response-like element composed of a GTT repeat and an intronic CCCCT repeat", "journal": "Genomics", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/8666386/" } ], "x": 7461.333333333333, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71189", "label": "AFMID hydrolyses NFK to L-KYN", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13010" ], "source_url": "https://reactome.org/content/detail/R-HSA-71189", "summary": "AFMID hydrolyses NFK to L-KYN is a Reactome release 97 curated reaction event. The source record connects NFK, H2O to L-KYN, HCOOH, H+ in cytosol and cross-references RHEA:13010.", "equation": "NFK + H2O → L-KYN + HCOOH + H+", "inputs": [ { "node_id": "R-ALL-33179", "reactome_id": "R-ALL-33179", "label": "NFK", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58629", "formula": "C11H12N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29970", "reactome_id": "R-ALL-29970", "label": "L-KYN", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57959", "formula": "C10H12N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29460", "reactome_id": "R-ALL-29460", "label": "HCOOH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15740", "formula": "CHO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "arylformamidase activity of AFMID [cytosol]", "activity_id": "178109", "ec_numbers": [ "3.5.1.9" ], "accessions": null } ], "citations": [ { "id": "PMID:12007602", "title": "Kynurenine formamidase: determination of primary structure and modeling-based prediction of tertiary structure and catalytic triad.", "journal": "Biochim Biophys Acta", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12007602/" } ], "x": 7461.333333333333, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71200", "label": "kynurenine + O2 + NADPH + H+ =\u003e 3-hydroxykynurenine + NADP+ + H2O", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:20546" ], "source_url": "https://reactome.org/content/detail/R-HSA-71200", "summary": "kynurenine + O2 + NADPH + H+ =\u003e 3-hydroxykynurenine + NADP+ + H2O is a Reactome release 97 curated reaction event. The source record connects L-KYN, O2, NADPH to 3HKYN, NADP+, H2O in cytosol and cross-references RHEA:20546.", "equation": "L-KYN + O2 + NADPH + H+ → 3HKYN + NADP+ + H2O", "inputs": [ { "node_id": "R-ALL-29970", "reactome_id": "R-ALL-29970", "label": "L-KYN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57959", "formula": "C10H12N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-34351", "reactome_id": "R-ALL-34351", "label": "3HKYN", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58125", "formula": "C10H12N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "kynurenine 3-monooxygenase activity of KMO [mitochondrial outer membrane]", "activity_id": "71199", "ec_numbers": [ "1.14.13.9" ], "accessions": null } ], "citations": [ { "id": "PMID:10672018", "title": "Functional characterization and mechanism of action of recombinant human kynurenine 3-hydroxylase", "journal": "Eur J Biochem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10672018/" }, { "id": "PMID:4291901", "title": "On the submitochondrial localization of l-kynurenine-3-hydroxylase", "journal": "Biochem Biophys Res Commun", "year": 1967, "url": "https://pubmed.ncbi.nlm.nih.gov/4291901/" } ], "x": 7740, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71217", "label": "3-hydroxykynurenine + H2O =\u003e 3-hydroxyanthranilate + alanine + H+", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:25144" ], "source_url": "https://reactome.org/content/detail/R-HSA-71217", "summary": "3-hydroxykynurenine + H2O =\u003e 3-hydroxyanthranilate + alanine + H+ is a Reactome release 97 curated reaction event. The source record connects 3HKYN, H2O to 3HAA, L-Ala, H+ in cytosol and cross-references RHEA:25144.", "equation": "3HKYN + H2O → 3HAA + L-Ala + H+", "inputs": [ { "node_id": "R-ALL-34351", "reactome_id": "R-ALL-34351", "label": "3HKYN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58125", "formula": "C10H12N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-30487", "reactome_id": "R-ALL-30487", "label": "3HAA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:36559", "formula": "C7H6NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29432", "reactome_id": "R-ALL-29432", "label": "L-Ala", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57972", "formula": "C3H7NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "kynureninase activity of KYNU dimer [cytosol]", "activity_id": "71216", "ec_numbers": [ "3.7.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:9180257", "title": "Cloning and recombinant expression of rat and human kynureninase.", "journal": "FEBS Lett", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9180257/" } ], "x": 7740, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71218", "label": "3-hydroxyanthranilate + O2 =\u003e 2-amino-3-carboxymuconate semialdehyde", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17954" ], "source_url": "https://reactome.org/content/detail/R-HSA-71218", "summary": "3-hydroxyanthranilate + O2 =\u003e 2-amino-3-carboxymuconate semialdehyde is a Reactome release 97 curated reaction event. The source record connects 3HAA, O2 to ACS in cytosol and cross-references RHEA:17954.", "equation": "3HAA + O2 → ACS", "inputs": [ { "node_id": "R-ALL-30487", "reactome_id": "R-ALL-30487", "label": "3HAA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36559", "formula": "C7H6NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-36027", "reactome_id": "R-ALL-36027", "label": "ACS", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:77612", "formula": "C7H6NO5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "3-hydroxyanthranilate 3,4-dioxygenase activity of HAAO [cytosol]", "activity_id": "71096", "ec_numbers": [ "1.13.11.6" ], "accessions": null } ], "citations": [ { "id": "PMID:7514594", "title": "Molecular cloning and functional expression of human 3-hydroxyanthranilic-acid dioxygenase.", "journal": "J Biol Chem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7514594/" } ], "x": 7740, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71239", "label": "2-aminomuconate semialdehyde + NAD+ + H2O =\u003e aminomuconate + NADH + H+", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14470" ], "source_url": "https://reactome.org/content/detail/R-HSA-71239", "summary": "2-aminomuconate semialdehyde + NAD+ + H2O =\u003e aminomuconate + NADH + H+ is a Reactome release 97 curated reaction event. The source record connects 2AMA, NAD+, H2O to 2AM, NADH, H+ in cytosol and cross-references RHEA:14470.", "equation": "2AMA + NAD+ + H2O → 2AM + NADH + H+", "inputs": [ { "node_id": "R-ALL-35181", "reactome_id": "R-ALL-35181", "label": "2AMA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:77634", "formula": "C6H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-32913", "reactome_id": "R-ALL-32913", "label": "2AM", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:77859", "formula": "C6H6NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aminomuconate-semialdehyde dehydrogenase activity of AM-SHDH [cytosol]", "activity_id": "71238", "ec_numbers": [ "1.2.1.32" ], "accessions": null } ], "citations": [ { "id": "PMID:14275130", "title": "Studies on the metabolism of the benzene ring of tryptophan in mammalian tissues. II. Enzymatic formation of alpha-muconic acid from 2-hydroxyanthranilic acid", "journal": "J Biol Chem", "year": 1965, "url": "https://pubmed.ncbi.nlm.nih.gov/14275130/" } ], "x": 8018.666666666667, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71296", "label": "PGLS hydrolyzes D-glucono-1,5-lactone 6-phosphate", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:12557" ], "source_url": "https://reactome.org/content/detail/R-HSA-71296", "summary": "PGLS hydrolyzes D-glucono-1,5-lactone 6-phosphate is a Reactome release 97 curated reaction event. The source record connects D-Glucono-1,5-lactone 6-phosphate, H2O to PDG, H+ in cytosol and cross-references RHEA:12557.", "equation": "D-Glucono-1,5-lactone 6-phosphate + H2O → PDG + H+", "inputs": [ { "node_id": "R-ALL-31467", "reactome_id": "R-ALL-31467", "label": "D-Glucono-1,5-lactone 6-phosphate", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57955", "formula": "C6H9O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29996", "reactome_id": "R-ALL-29996", "label": "PDG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58759", "formula": "C6H10O10P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "6-phosphogluconolactonase activity of PGLS [cytosol]", "activity_id": "71295", "ec_numbers": [ "3.1.1.31" ], "accessions": null } ], "citations": [ { "id": "PMID:3932573", "title": "Limiting role of 6-phosphogluconolactonase in erythrocyte hexose monophosphate pathway metabolism", "journal": "J Lab Clin Med", "year": 1985, "url": "https://pubmed.ncbi.nlm.nih.gov/3932573/" }, { "id": "PMID:10518023", "title": "Identification of the cDNA encoding human 6-phosphogluconolactonase, the enzyme catalyzing the second step of the pentose phosphate pathway(1)", "journal": "FEBS Lett", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10518023/" } ], "x": 311.33333333333337, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71299", "label": "PGD decarboxylates 6-phospho-D-gluconate", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10117" ], "source_url": "https://reactome.org/content/detail/R-HSA-71299", "summary": "PGD decarboxylates 6-phospho-D-gluconate is a Reactome release 97 curated reaction event. The source record connects PDG, NADP+ to RU5P, CO2, NADPH in cytosol and cross-references RHEA:10117.", "equation": "PDG + NADP+ → RU5P + CO2 + NADPH", "inputs": [ { "node_id": "R-ALL-29996", "reactome_id": "R-ALL-29996", "label": "PDG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58759", "formula": "C6H10O10P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29732", "reactome_id": "R-ALL-29732", "label": "RU5P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58121", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphogluconate dehydrogenase (decarboxylating) activity of PGD dimer [cytosol]", "activity_id": "71298", "ec_numbers": [ "1.1.1.44" ], "accessions": null } ], "citations": [ { "id": "PMID:3932573", "title": "Limiting role of 6-phosphogluconolactonase in erythrocyte hexose monophosphate pathway metabolism", "journal": "J Lab Clin Med", "year": 1985, "url": "https://pubmed.ncbi.nlm.nih.gov/3932573/" }, { "id": "PMID:3994686", "title": "Half-site reactivity in 6-phosphogluconate dehydrogenase from human erythrocytes", "journal": "Biochem J", "year": 1985, "url": "https://pubmed.ncbi.nlm.nih.gov/3994686/" }, { "id": "PMID:9920387", "title": "6-Phosphogluconate dehydrogenase: the mechanism of action investigated by a comparison of the enzyme from different species", "journal": "Biochim Biophys Acta", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9920387/" } ], "x": 311.33333333333337, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71334", "label": "sedoheptulose 7-phosphate + D-glyceraldehyde 3-phosphate D-erythrose 4-phosphate + D-fructose 6-phosphate", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17054" ], "source_url": "https://reactome.org/content/detail/R-HSA-71334", "summary": "sedoheptulose 7-phosphate + D-glyceraldehyde 3-phosphate D-erythrose 4-phosphate + D-fructose 6-phosphate is a Reactome release 97 curated reaction event. The source record connects SH7P, GA3P to Fru(6)P, E4P in cytosol and cross-references RHEA:17054.", "equation": "SH7P + GA3P → Fru(6)P + E4P", "inputs": [ { "node_id": "R-ALL-29882", "reactome_id": "R-ALL-29882", "label": "SH7P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57483", "formula": "C7H13O10P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29878", "reactome_id": "R-ALL-29878", "label": "E4P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16897", "formula": "C4H7O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "transaldolase activity of TALDO1 dimer [cytosol]", "activity_id": "71333", "ec_numbers": [ "2.2.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:8300619", "title": "Cloning and expression of the human gene for transaldolase. A novel highly repetitive element constitutes an integral part of the coding sequence", "journal": "J Biol Chem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8300619/" }, { "id": "PMID:10869557", "title": "The three-dimensional structure of human transaldolase", "journal": "FEBS Lett.", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10869557/" }, { "id": "PMID:11283793", "title": "Transaldolase deficiency: liver cirrhosis associated with a new inborn error in the pentose phosphate pathway.", "journal": "Am J Hum Genet", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11283793/" } ], "x": 590, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71335", "label": "xylulose 5-phosphate + D-erythrose 4-phosphate D-glyceraldehyde 3-phosphate + D-fructose 6-phosphate", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:27627" ], "source_url": "https://reactome.org/content/detail/R-HSA-71335", "summary": "xylulose 5-phosphate + D-erythrose 4-phosphate D-glyceraldehyde 3-phosphate + D-fructose 6-phosphate is a Reactome release 97 curated reaction event. The source record connects XY5P, E4P to GA3P, Fru(6)P in cytosol and cross-references RHEA:27627.", "equation": "XY5P + E4P → GA3P + Fru(6)P", "inputs": [ { "node_id": "R-ALL-29790", "reactome_id": "R-ALL-29790", "label": "XY5P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57737", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29878", "reactome_id": "R-ALL-29878", "label": "E4P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16897", "formula": "C4H7O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "transketolase activity of TKT dimer [cytosol]", "activity_id": "71323", "ec_numbers": [ "2.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:9357955", "title": "Aspartate 155 of human transketolase is essential for thiamine diphosphate-magnesium binding, and cofactor binding is required for dimer formation", "journal": "Biochim Biophys Acta", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9357955/" } ], "x": 868.6666666666667, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71401", "label": "OGDH dimer decarboxylates 2-OG", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:12189" ], "source_url": "https://reactome.org/content/detail/R-HSA-71401", "summary": "OGDH dimer decarboxylates 2-OG is a Reactome release 97 curated reaction event. The source record connects 2OG, H+, Lipo-K110-DLST to CO2, SuccinyllipoH2-K110-DLST in mitochondrial matrix and cross-references RHEA:12189.", "equation": "2OG + H+ + Lipo-K110-DLST → CO2 + SuccinyllipoH2-K110-DLST", "inputs": [ { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-HSA-69993", "reactome_id": "R-HSA-69993", "label": "Lipo-K110-DLST", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-HSA-9853502", "reactome_id": "R-HSA-9853502", "label": "SuccinyllipoH2-K110-DLST", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "catalysts": [ { "label": "oxoglutarate dehydrogenase (succinyl-transferring) activity of OGDH complex [mitochondrial matrix]", "activity_id": "9859269", "ec_numbers": [ "1.2.4.2" ], "accessions": null } ], "citations": [ { "id": "PMID:35272141", "title": "Structural basis for the activity and regulation of human α-ketoglutarate dehydrogenase revealed by Cryo-EM", "journal": "Biochem Biophys Res Commun", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/35272141/" }, { "id": "PMID:28435050", "title": "The human Krebs cycle 2-oxoglutarate dehydrogenase complex creates an additional source of superoxide/hydrogen peroxide from 2-oxoadipate as alternative substrate", "journal": "Free Radic Biol Med", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28435050/" }, { "id": "PMID:25210035", "title": "Human 2-oxoglutarate dehydrogenase complex E1 component forms a thiamin-derived radical by aerobic oxidation of the enamine intermediate", "journal": "J Biol Chem", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/25210035/" }, { "id": "PMID:24495017", "title": "Studies on the regulation of the human E1 subunit of the 2-oxoglutarate dehydrogenase complex, including the identification of a novel calcium-binding site", "journal": "Biochem J", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24495017/" } ], "x": 5948.666666666667, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71445", "label": "PGAM dimers isomerise 2PG to 3PG", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15902" ], "source_url": "https://reactome.org/content/detail/R-HSA-71445", "summary": "PGAM dimers isomerise 2PG to 3PG is a Reactome release 97 curated reaction event. The source record connects 2PG to 3PG in cytosol and cross-references RHEA:15902.", "equation": "2PG → 3PG", "inputs": [ { "node_id": "R-ALL-30485", "reactome_id": "R-ALL-30485", "label": "2PG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58289", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29728", "reactome_id": "R-ALL-29728", "label": "3PG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58272", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phosphoglycerate mutase activity of PGAM dimers [cytosol]", "activity_id": "71444", "ec_numbers": [ "5.4.2.11" ], "accessions": null } ], "citations": [ { "id": "PMID:4827367", "title": "Phosphoglycerate mutase isozyme marker for tissue differentiation in man", "journal": "Am J Hum Genet", "year": 1974, "url": "https://pubmed.ncbi.nlm.nih.gov/4827367/" }, { "id": "PMID:15710582", "title": "Phosphoglycerate mutase BB isoenzyme deficiency in a patient with non-spherocytic anemia: familial and metabolic studies", "journal": "Haematologica", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15710582/" }, { "id": "PMID:8447317", "title": "The molecular genetic basis of muscle phosphoglycerate mutase (PGAM) deficiency", "journal": "Am J Hum Genet", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8447317/" } ], "x": 1530, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71495", "label": "Aldolase tetramers convert GA3P and DHAP to F1,6PP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "gluconeogenesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14731" ], "source_url": "https://reactome.org/content/detail/R-HSA-71495", "summary": "Aldolase tetramers convert GA3P and DHAP to F1,6PP is a Reactome release 97 curated reaction event. The source record connects DHAP, GA3P to F1,6PP in cytosol and cross-references RHEA:14731.", "equation": "DHAP + GA3P → F1,6PP", "inputs": [ { "node_id": "R-ALL-75970", "reactome_id": "R-ALL-75970", "label": "DHAP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57642", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-30014", "reactome_id": "R-ALL-30014", "label": "F1,6PP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:32966", "formula": "C6H10O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "fructose-bisphosphate aldolase activity of aldolase tetramer [cytosol]", "activity_id": "71494", "ec_numbers": [ "4.1.2.13" ], "accessions": null } ], "citations": [ { "id": "PMID:7717389", "title": "Diverse mutations in the aldolase B gene that underlie the prevalence of hereditary fructose intolerance", "journal": "Am J Hum Genet", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7717389/" }, { "id": "PMID:3355497", "title": "The complete amino acid sequence of human skeletal-muscle fructose-bisphosphate aldolase", "journal": "Biochem J", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3355497/" } ], "x": 1530, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71496", "label": "Aldolase tetramer cleaves F1,6PP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14730" ], "source_url": "https://reactome.org/content/detail/R-HSA-71496", "summary": "Aldolase tetramer cleaves F1,6PP is a Reactome release 97 curated reaction event. The source record connects F1,6PP to DHAP, GA3P in cytosol and cross-references RHEA:14730.", "equation": "F1,6PP → DHAP + GA3P", "inputs": [ { "node_id": "R-ALL-30014", "reactome_id": "R-ALL-30014", "label": "F1,6PP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:32966", "formula": "C6H10O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-75970", "reactome_id": "R-ALL-75970", "label": "DHAP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57642", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29578", "reactome_id": "R-ALL-29578", "label": "GA3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:59776", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "fructose-bisphosphate aldolase activity of aldolase tetramer [cytosol]", "activity_id": "71494", "ec_numbers": [ "4.1.2.13" ], "accessions": null } ], "citations": [ { "id": "PMID:7717389", "title": "Diverse mutations in the aldolase B gene that underlie the prevalence of hereditary fructose intolerance", "journal": "Am J Hum Genet", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7717389/" }, { "id": "PMID:6696436", "title": "Human skeletal-muscle aldolase: N-terminal sequence analysis of CNBr- and o-iodosobenzoic acid-cleavage fragments", "journal": "Arch Biochem Biophys", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6696436/" }, { "id": "PMID:3355497", "title": "The complete amino acid sequence of human skeletal-muscle fructose-bisphosphate aldolase", "journal": "Biochem J", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3355497/" } ], "x": 311.33333333333337, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71660", "label": "Enolase dimers dehydrates 2PG", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10165" ], "source_url": "https://reactome.org/content/detail/R-HSA-71660", "summary": "Enolase dimers dehydrates 2PG is a Reactome release 97 curated reaction event. The source record connects 2PG to PEP, H2O in cytosol and cross-references RHEA:10165.", "equation": "2PG → PEP + H2O", "inputs": [ { "node_id": "R-ALL-30485", "reactome_id": "R-ALL-30485", "label": "2PG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58289", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29492", "reactome_id": "R-ALL-29492", "label": "PEP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58702", "formula": "C3H2O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphopyruvate hydratase activity of enolase dimer [cytosol]", "activity_id": "71439", "ec_numbers": [ "4.2.1.11" ], "accessions": null } ], "citations": [ { "id": "PMID:11506403", "title": "Beta-enolase deficiency, a new metabolic myopathy of distal glycolysis", "journal": "Ann Neurol", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11506403/" }, { "id": "PMID:3529090", "title": "Molecular cloning and nucleotide sequence of a full-length cDNA for human alpha enolase", "journal": "Proc Natl Acad Sci U S A", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3529090/" }, { "id": "PMID:3208766", "title": "Complete amino acid sequence of the neurone-specific gamma isozyme of enolase (NSE) from human brain and comparison with the non-neuronal alpha form (NNE)", "journal": "Eur J Biochem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3208766/" }, { "id": "PMID:23446454", "title": "Disruption of a spermatogenic cell-specific mouse enolase 4 (eno4) gene causes sperm structural defects and male infertility", "journal": "Biol Reprod", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23446454/" }, { "id": "PMID:4413246", "title": "Enolase isoenzymes in rat tissues. Electrophoretic, chromatographic, immunological and kinetic properties", "journal": "Biochim Biophys Acta", "year": 1974, "url": "https://pubmed.ncbi.nlm.nih.gov/4413246/" }, { "id": "PMID:2462567", "title": "Tau-crystallin/alpha-enolase: one gene encodes both an enzyme and a lens structural protein", "journal": "J Cell Biol", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/2462567/" } ], "x": 590, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71670", "label": "Pyruvate kinase dephosphorylates PEP to PYR", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis", "pyruvate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18159" ], "source_url": "https://reactome.org/content/detail/R-HSA-71670", "summary": "Pyruvate kinase dephosphorylates PEP to PYR is a Reactome release 97 curated reaction event. The source record connects ADP, PEP, H+ to ATP, PYR in cytosol and cross-references RHEA:18159.", "equation": "ADP + PEP + H+ → ATP + PYR", "inputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29492", "reactome_id": "R-ALL-29492", "label": "PEP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58702", "formula": "C3H2O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "pyruvate kinase activity of pyruvate kinase tetramer [cytosol]", "activity_id": "71669", "ec_numbers": [ "2.7.1.40" ], "accessions": null } ], "citations": [ { "id": "PMID:15996096", "title": "Structural basis for tumor pyruvate kinase M2 allosteric regulation and catalysis", "journal": "Biochemistry", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15996096/" }, { "id": "PMID:1896471", "title": "cDNA cloning of human R-type pyruvate kinase and identification of a single amino acid substitution (Thr384----Met) affecting enzymatic stability in a pyruvate kinase variant (PK Tokyo) associated with hereditary hemolytic anemia", "journal": "Proc Natl Acad Sci U S A", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1896471/" }, { "id": "PMID:18191611", "title": "Pyruvate kinase isozyme type M2 (PKM2) interacts and cooperates with Oct-4 in regulating transcription", "journal": "Int J Biochem Cell Biol", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18191611/" }, { "id": "PMID:2040271", "title": "Isolation and characterization of the human pyruvate kinase M gene", "journal": "Eur J Biochem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/2040271/" }, { "id": "PMID:3126495", "title": "Human liver type pyruvate kinase: complete amino acid sequence and the expression in mammalian cells", "journal": "Proc Natl Acad Sci U S A", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3126495/" }, { "id": "PMID:11960989", "title": "Structure and function of human erythrocyte pyruvate kinase. Molecular basis of nonspherocytic hemolytic anemia", "journal": "J Biol Chem", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11960989/" } ], "x": 868.6666666666667, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71775", "label": "SUCLG1/G2 cleaves succinyl-CoA", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:22122" ], "source_url": "https://reactome.org/content/detail/R-HSA-71775", "summary": "SUCLG1/G2 cleaves succinyl-CoA is a Reactome release 97 curated reaction event. The source record connects GDP, Pi, SUCC-CoA to GTP, SUCCA, CoA-SH in mitochondrial matrix and cross-references RHEA:22122.", "equation": "GDP + Pi + SUCC-CoA → GTP + SUCCA + CoA-SH", "inputs": [ { "node_id": "R-ALL-113525", "reactome_id": "R-ALL-113525", "label": "GDP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58189", "formula": "C10H12N5O11P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113548", "reactome_id": "R-ALL-113548", "label": "Pi", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70958", "reactome_id": "R-ALL-70958", "label": "SUCC-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57292", "formula": "C25H35N7O19P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113573", "reactome_id": "R-ALL-113573", "label": "GTP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:37565", "formula": "C10H12N5O14P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113536", "reactome_id": "R-ALL-113536", "label": "SUCCA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30031", "formula": "C4H4O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "succinate-CoA ligase (GDP-forming) activity of SUCLG1:SUCLG2 [mitochondrial matrix]", "activity_id": "71774", "ec_numbers": [ "6.2.1.4" ], "accessions": null } ], "citations": [ { "id": "PMID:10727444", "title": "Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia", "journal": "J Clin Invest", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10727444/" }, { "id": "PMID:9765291", "title": "Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9765291/" }, { "id": "PMID:9765290", "title": "Characterization of the ATP- and GTP-specific succinyl-CoA synthetases in pigeon. The enzymes incorporate the same alpha-subunit.", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9765290/" }, { "id": "PMID:15234968", "title": "Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15234968/" }, { "id": "PMID:17668387", "title": "Deficiency of the alpha subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion", "journal": "Am J Hum Genet", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17668387/" }, { "id": "PMID:13181903", "title": "Guanosine triphosphate, the primary product of phosphorylation coupled to the breakdown of succinyl coenzyme A", "journal": "Biochim Biophys Acta", "year": 1954, "url": "https://pubmed.ncbi.nlm.nih.gov/13181903/" } ], "x": 5670, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71802", "label": "PFKFKB dimer phosphorylates Fru(6)P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15654" ], "source_url": "https://reactome.org/content/detail/R-HSA-71802", "summary": "PFKFKB dimer phosphorylates Fru(6)P is a Reactome release 97 curated reaction event. The source record connects Fru(6)P, ATP to D-Fructose 2,6-bisphosphate, ADP, H+ in cytosol and cross-references RHEA:15654.", "equation": "Fru(6)P + ATP → D-Fructose 2,6-bisphosphate + ADP + H+", "inputs": [ { "node_id": "R-ALL-29512", "reactome_id": "R-ALL-29512", "label": "Fru(6)P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57634", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-30533", "reactome_id": "R-ALL-30533", "label": "D-Fructose 2,6-bisphosphate", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58579", "formula": "C6H10O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "6-phosphofructo-2-kinase activity of PFKFB dimers [cytosol]", "activity_id": "163768", "ec_numbers": [ "2.7.1.105" ], "accessions": null } ], "citations": [ { "id": "PMID:2837207", "title": "Molecular cloning, sequence analysis, and expression of a human liver cDNA coding for fructose-6-P,2-kinase:fructose-2,6-bisphosphatase", "journal": "Biochem Biophys Res Commun", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/2837207/" }, { "id": "PMID:9464277", "title": "Expression of human placental-type 6-phosphofructo-2-kinase/fructose 2,6-bisphosphatase in various cells and cell lines", "journal": "Biochem Biophys Res Commun", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9464277/" }, { "id": "PMID:15896703", "title": "The kinase activity of human brain 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase is regulated via inhibition by phosphoenolpyruvate", "journal": "Arch Biochem Biophys", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15896703/" }, { "id": "PMID:10095107", "title": "Cloning, expression and chromosomal localization of a human testis 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase gene", "journal": "Gene", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10095107/" }, { "id": "PMID:3052289", "title": "Hormonal regulation of hepatic gluconeogenesis and glycolysis", "journal": "Annu Rev Biochem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3052289/" }, { "id": "PMID:7574501", "title": "6-Phosphofructo-2-kinase/fructose-2,6-bisphosphatase: a metabolic signaling enzyme.", "journal": "Annu Rev Biochem", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7574501/" } ], "x": 311.33333333333337, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71849", "label": "LDH tetramer reduces PYR to LACT", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23446" ], "source_url": "https://reactome.org/content/detail/R-HSA-71849", "summary": "LDH tetramer reduces PYR to LACT is a Reactome release 97 curated reaction event. The source record connects PYR, NADH, H+ to LACT, NAD+ in cytosol and cross-references RHEA:23446.", "equation": "PYR + NADH + H+ → LACT + NAD+", "inputs": [ { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29708", "reactome_id": "R-ALL-29708", "label": "LACT", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16651", "formula": "C3H5O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-lactate dehydrogenase (NAD+) activity of LDH tetramer [cytosol]", "activity_id": "70509", "ec_numbers": [ "1.1.1.27" ], "accessions": null } ], "citations": [ { "id": "PMID:18351441", "title": "Identification of a novel human lactate dehydrogenase gene LDHAL6A, which activates transcriptional activities of AP1(PMA)", "journal": "Mol. Biol. Rep.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/18351441/" }, { "id": "PMID:1996957", "title": "Properties of human testis-specific lactate dehydrogenase expressed from Escherichia coli", "journal": "Biochem. J.", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1996957/" }, { "id": "PMID:2440048", "title": "Epitopes of human testis-specific lactate dehydrogenase deduced from a cDNA sequence", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/2440048/" }, { "id": "PMID:11276087", "title": "Structural basis for altered activity of M- and H-isozyme forms of human lactate dehydrogenase", "journal": "Proteins", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11276087/" }, { "id": "PMID:3435492", "title": "The cDNA and protein sequences of human lactate dehydrogenase B", "journal": "Biochem J", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3435492/" }, { "id": "PMID:11377399", "title": "Selective active site inhibitors of human lactate dehydrogenases A4, B4, and C4", "journal": "Biochem Pharmacol", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11377399/" } ], "x": 4451.333333333333, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-71850", "label": "PGK complexes dephosphorylate 1,3BPG", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14803" ], "source_url": "https://reactome.org/content/detail/R-HSA-71850", "summary": "PGK complexes dephosphorylate 1,3BPG is a Reactome release 97 curated reaction event. The source record connects 1,3BPG, ADP to 3PG, ATP in cytosol and cross-references RHEA:14803.", "equation": "1,3BPG + ADP → 3PG + ATP", "inputs": [ { "node_id": "R-ALL-29800", "reactome_id": "R-ALL-29800", "label": "1,3BPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57604", "formula": "C3H4O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29728", "reactome_id": "R-ALL-29728", "label": "3PG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58272", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoglycerate kinase activity of PGK complexes [cytosol]", "activity_id": "70485", "ec_numbers": [ "2.7.2.3" ], "accessions": null } ], "citations": [ { "id": "PMID:1278465", "title": "Characterization of phosphoglycerate kinase from human spermatozoa", "journal": "Fertil. Steril.", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/1278465/" }, { "id": "PMID:6771269", "title": "Complete amino acid sequence of human phosphoglycerate kinase. Isolation and amino acid sequence of tryptic peptides.", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6771269/" }, { "id": "PMID:7391027", "title": "Complete amino acid sequence of human phosphoglycerate kinase. Cyanogen bromide peptides and complete amino acid sequence.", "journal": "J Biol Chem", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/7391027/" }, { "id": "PMID:3453121", "title": "Human testis-specific PGK gene lacks introns and possesses characteristics of a processed gene", "journal": "Nature", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3453121/" }, { "id": "PMID:5009693", "title": "Human phosphoglycerate kinase. I. Crystallization and characterization of normal enzyme", "journal": "J Biol Chem", "year": 1972, "url": "https://pubmed.ncbi.nlm.nih.gov/5009693/" } ], "x": 590, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73564", "label": "UMPS dimer decarboxylates OMP to UMP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11597" ], "source_url": "https://reactome.org/content/detail/R-HSA-73564", "summary": "UMPS dimer decarboxylates OMP to UMP is a Reactome release 97 curated reaction event. The source record connects OMP, H+ to UMP, CO2 in cytosol and cross-references RHEA:11597.", "equation": "OMP + H+ → UMP + CO2", "inputs": [ { "node_id": "R-ALL-111629", "reactome_id": "R-ALL-111629", "label": "OMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57538", "formula": "C10H10N2O11P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-109402", "reactome_id": "R-ALL-109402", "label": "UMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57865", "formula": "C9H11N2O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "orotidine-5'-phosphate decarboxylase activity of UMPS dimer [cytosol]", "activity_id": "73492", "ec_numbers": [ "4.1.1.23" ], "accessions": null } ], "citations": [ { "id": "PMID:9042911", "title": "Molecular cloning of the human UMP synthase gene and characterization of point mutations in two hereditary orotic aciduria families", "journal": "Am J Hum Genet", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9042911/" }, { "id": "PMID:8631878", "title": "Intrinsic activity and stability of bifunctional human UMP synthase and its two separate catalytic domains, orotate phosphoribosyltransferase and orotidine-5'-phosphate decarboxylase", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8631878/" }, { "id": "PMID:18184586", "title": "Structures of the human orotidine-5'-monophosphate decarboxylase support a covalent mechanism and provide a framework for drug design", "journal": "Structure", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18184586/" }, { "id": "PMID:6893554", "title": "Isolation and initial characterization of the single polypeptide that synthesizes uridine 5'-monophosphate from orotate in Ehrlich ascites carcinoma. Purification by tandem affinity chromatography of uridine-5'-monophosphate synthase", "journal": "Biochemistry", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6893554/" } ], "x": 2660, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73567", "label": "UMPS dimer transfers phosphoribosyl group to ORO to form OMP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10382" ], "source_url": "https://reactome.org/content/detail/R-HSA-73567", "summary": "UMPS dimer transfers phosphoribosyl group to ORO to form OMP is a Reactome release 97 curated reaction event. The source record connects ORO, PRPP to OMP, PPi in cytosol and cross-references RHEA:10382.", "equation": "ORO + PRPP → OMP + PPi", "inputs": [ { "node_id": "R-ALL-76632", "reactome_id": "R-ALL-76632", "label": "ORO", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30839", "formula": "C5H3N2O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73566", "reactome_id": "R-ALL-73566", "label": "PRPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58017", "formula": "C5H8O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-111629", "reactome_id": "R-ALL-111629", "label": "OMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57538", "formula": "C10H10N2O11P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "orotate phosphoribosyltransferase activity of UMPS dimer [cytosol]", "activity_id": "73491", "ec_numbers": [ "2.4.2.10" ], "accessions": null } ], "citations": [ { "id": "PMID:9042911", "title": "Molecular cloning of the human UMP synthase gene and characterization of point mutations in two hereditary orotic aciduria families", "journal": "Am J Hum Genet", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9042911/" }, { "id": "PMID:8631878", "title": "Intrinsic activity and stability of bifunctional human UMP synthase and its two separate catalytic domains, orotate phosphoribosyltransferase and orotidine-5'-phosphate decarboxylase", "journal": "J Biol Chem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8631878/" }, { "id": "PMID:6893554", "title": "Isolation and initial characterization of the single polypeptide that synthesizes uridine 5'-monophosphate from orotate in Ehrlich ascites carcinoma. Purification by tandem affinity chromatography of uridine-5'-monophosphate synthase", "journal": "Biochemistry", "year": 1980, "url": "https://pubmed.ncbi.nlm.nih.gov/6893554/" }, { "id": "PMID:18184586", "title": "Structures of the human orotidine-5'-monophosphate decarboxylase support a covalent mechanism and provide a framework for drug design", "journal": "Structure", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18184586/" } ], "x": 2938.666666666667, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73573", "label": "CAD hexamer transforms CAP to N-carb-L-Asp", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:20014" ], "source_url": "https://reactome.org/content/detail/R-HSA-73573", "summary": "CAD hexamer transforms CAP to N-carb-L-Asp is a Reactome release 97 curated reaction event. The source record connects CAP, L-Asp to N-carb-L-Asp, Pi, H+ in cytosol and cross-references RHEA:20014.", "equation": "CAP + L-Asp → N-carb-L-Asp + Pi + H+", "inputs": [ { "node_id": "R-ALL-111647", "reactome_id": "R-ALL-111647", "label": "CAP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58228", "formula": "CH2NO5P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29448", "reactome_id": "R-ALL-29448", "label": "L-Asp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-111628", "reactome_id": "R-ALL-111628", "label": "N-carb-L-Asp", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:32814", "formula": "C5H6N2O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aspartate carbamoyltransferase activity of CAD hexamer [cytosol]", "activity_id": "73459", "ec_numbers": [ "2.1.3.2" ], "accessions": null } ], "citations": [ { "id": "PMID:4684686", "title": "Control of pyrimidine biosynthesis in human lymphocytes. Simultaneous increase in activities of glutamine-utilizing carbamyl phosphate synthetase and aspartate transcarbamylase in phytohemagglutinin-stimulated human peripheral lymphocytes and their enzyme co-purification", "journal": "J Biol Chem", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4684686/" }, { "id": "PMID:8619816", "title": "Molecular cloning of a human cDNA encoding a trifunctional enzyme of carbamoyl-phosphate synthetase-aspartate transcarbamoylase-dihydroorotase in de Novo pyrimidine synthesis", "journal": "Biochem Biophys Res Commun", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8619816/" }, { "id": "PMID:2995985", "title": "Oligomeric structure of the multifunctional protein CAD that initiates pyrimidine biosynthesis in mammalian cells", "journal": "Proc Natl Acad Sci USA", "year": 1985, "url": "https://pubmed.ncbi.nlm.nih.gov/2995985/" } ], "x": 2381.3333333333335, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73577", "label": "CAD hexamer transforms L-Gln to CAP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18634" ], "source_url": "https://reactome.org/content/detail/R-HSA-73577", "summary": "CAD hexamer transforms L-Gln to CAP is a Reactome release 97 curated reaction event. The source record connects HCO3-, L-Gln, ATP to CAP, L-Glu, ADP in cytosol and cross-references RHEA:18634.", "equation": "HCO3- + L-Gln + 2 ATP + H2O → CAP + L-Glu + 2 ADP + Pi + 2 H+", "inputs": [ { "node_id": "R-ALL-111627", "reactome_id": "R-ALL-111627", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29472", "reactome_id": "R-ALL-29472", "label": "L-Gln", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58359", "formula": "C5H10N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111647", "reactome_id": "R-ALL-111647", "label": "CAP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58228", "formula": "CH2NO5P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity of CAD hexamer [cytosol]", "activity_id": "73458", "ec_numbers": [ "6.3.5.5" ], "accessions": null } ], "citations": [ { "id": "PMID:4684686", "title": "Control of pyrimidine biosynthesis in human lymphocytes. Simultaneous increase in activities of glutamine-utilizing carbamyl phosphate synthetase and aspartate transcarbamylase in phytohemagglutinin-stimulated human peripheral lymphocytes and their enzyme co-purification", "journal": "J Biol Chem", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4684686/" }, { "id": "PMID:8619816", "title": "Molecular cloning of a human cDNA encoding a trifunctional enzyme of carbamoyl-phosphate synthetase-aspartate transcarbamoylase-dihydroorotase in de Novo pyrimidine synthesis", "journal": "Biochem Biophys Res Commun", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8619816/" } ], "x": 2381.3333333333335, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73580", "label": "PRPS dimers pyrophosphorylate R5P using ATP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15610" ], "source_url": "https://reactome.org/content/detail/R-HSA-73580", "summary": "PRPS dimers pyrophosphorylate R5P using ATP is a Reactome release 97 curated reaction event. The source record connects R5P, ATP to PRPP, AMP, H+ in cytosol and cross-references RHEA:15610.", "equation": "R5P + ATP → PRPP + AMP + H+", "inputs": [ { "node_id": "R-ALL-73578", "reactome_id": "R-ALL-73578", "label": "R5P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:78346", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-73566", "reactome_id": "R-ALL-73566", "label": "PRPP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58017", "formula": "C5H8O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ribose phosphate diphosphokinase activity of PRPS dimers [cytosol]", "activity_id": "111174", "ec_numbers": [ "2.7.6.1" ], "accessions": null } ], "citations": [ { "id": "PMID:4328836", "title": "Human phosphoribosylpyrophosphate synthetase", "journal": "J Biol Chem", "year": 1971, "url": "https://pubmed.ncbi.nlm.nih.gov/4328836/" }, { "id": "PMID:28031352", "title": "Phosphoribosyl Diphosphate (PRPP): Biosynthesis, Enzymology, Utilization, and Metabolic Significance", "journal": "Microbiol Mol Biol Rev", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28031352/" } ], "x": 590, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73598", "label": "(2'-deoxy)cytidine + ATP =\u003e (d)CMP + ADP (DCK)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:46041", "RHEA:24675" ], "source_url": "https://reactome.org/content/detail/R-HSA-73598", "summary": "(2'-deoxy)cytidine + ATP =\u003e (d)CMP + ADP (DCK) is a Reactome release 97 curated reaction event. The source record connects Cyt-Rib, dC, ATP to (d)CMP, ADP, H+ in cytosol and cross-references RHEA:46041, RHEA:24675.", "equation": "Cyt-Rib, dC + ATP → (d)CMP + ADP + H+", "inputs": [ { "node_id": "R-ALL-500829", "reactome_id": "R-ALL-500829", "label": "Cyt-Rib, dC", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500828", "reactome_id": "R-ALL-500828", "label": "(d)CMP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside kinase activity of DCK dimer [cytosol]", "activity_id": "73517", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:2844225", "title": "Deoxycytidine kinase from human leukemic spleen: preparation and characterization of the homogeneous enzyme", "journal": "Biochemistry", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/2844225/" }, { "id": "PMID:1996353", "title": "Cloning and expression of human deoxycytidine kinase cDNA", "journal": "Proc Natl Acad Sci USA", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1996353/" }, { "id": "PMID:2539852", "title": "Human T-lymphoblast deoxycytidine kinase: purification and properties", "journal": "Biochemistry", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2539852/" }, { "id": "PMID:2542307", "title": "Kinetic properties and inhibition of human T lymphoblast deoxycytidine kinase", "journal": "J Biol Chem", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2542307/" }, { "id": "PMID:9804782", "title": "The intracellular localization of deoxycytidine kinase", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9804782/" }, { "id": "PMID:12808445", "title": "Structure of human dCK suggests strategies to improve anticancer and antiviral therapy", "journal": "Nat Struct Biol", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12808445/" } ], "x": 3321.3333333333335, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73608", "label": "deamination of 2'-Deoxycytidine to 2'-Deoxyuridine", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13434", "RHEA:16070" ], "source_url": "https://reactome.org/content/detail/R-HSA-73608", "summary": "deamination of 2'-Deoxycytidine to 2'-Deoxyuridine is a Reactome release 97 curated reaction event. The source record connects Cyt-Rib, dC, H2O, H+ to (d)Ura, NH4+ in cytosol and cross-references RHEA:13434, RHEA:16070.", "equation": "Cyt-Rib, dC + H2O + H+ → (d)Ura + NH4+", "inputs": [ { "node_id": "R-ALL-500829", "reactome_id": "R-ALL-500829", "label": "Cyt-Rib, dC", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500430", "reactome_id": "R-ALL-500430", "label": "(d)Ura", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "cytidine deaminase activity of CDA tetramer [cytosol]", "activity_id": "73463", "ec_numbers": [ "3.5.4.5" ], "accessions": null } ], "citations": [ { "id": "PMID:15689149", "title": "Structure of human cytidine deaminase bound to a potent inhibitor", "journal": "J Med Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15689149/" }, { "id": "PMID:7923172", "title": "Human cytidine deaminase: purification of enzyme, cloning, and expression of its complementary DNA", "journal": "Cancer Res", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7923172/" } ], "x": 3600, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73632", "label": "phosphorylation of 2'-Deoxythymidine to dTMP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19130" ], "source_url": "https://reactome.org/content/detail/R-HSA-73632", "summary": "phosphorylation of 2'-Deoxythymidine to dTMP is a Reactome release 97 curated reaction event. The source record connects Thy-dRib, ATP to TMP, ADP, H+ in cytosol and cross-references RHEA:19130.", "equation": "Thy-dRib + ATP → TMP + ADP + H+", "inputs": [ { "node_id": "R-ALL-83928", "reactome_id": "R-ALL-83928", "label": "Thy-dRib", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17748", "formula": "C10H14N2O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-109366", "reactome_id": "R-ALL-109366", "label": "TMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:63528", "formula": "C10H13N2O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside kinase activity of TK1 tetramer [cytosol]", "activity_id": "73502", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:15733844", "title": "Structure of a type II thymidine kinase with bound dTTP", "journal": "FEBS Lett", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15733844/" }, { "id": "PMID:1063125", "title": "Human deoxythymidine kinase. I. Purification and general properties of the cytoplasmic and mitochondrial isozymes derived from blast cells of acute myelocytic leukemia", "journal": "J Biol Chem", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/1063125/" }, { "id": "PMID:2026611", "title": "Diverging substrate specificity of pure human thymidine kinases 1 and 2 against antiviral dideoxynucleosides", "journal": "J Biol Chem", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/2026611/" }, { "id": "PMID:8340387", "title": "Reversible ATP-dependent transition between two forms of human cytosolic thymidine kinase with different enzymatic properties", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8340387/" }, { "id": "PMID:3372530", "title": "Regulation of human thymidine kinase during the cell cycle", "journal": "J Biol Chem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3372530/" }, { "id": "PMID:3335503", "title": "Human cytosolic thymidine kinase. Purification and physical characterization of the enzyme from HeLa cells", "journal": "J Biol Chem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3335503/" } ], "x": 3600, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73792", "label": "XMP + L-Glutamine + ATP + H2O =\u003e GMP + L-Glutamate + AMP + pyrophosphate", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11681" ], "source_url": "https://reactome.org/content/detail/R-HSA-73792", "summary": "XMP + L-Glutamine + ATP + H2O =\u003e GMP + L-Glutamate + AMP + pyrophosphate is a Reactome release 97 curated reaction event. The source record connects XMP, L-Gln, ATP to GMP, L-Glu, H+ in cytosol and cross-references RHEA:11681.", "equation": "XMP + L-Gln + ATP + H2O → GMP + L-Glu + 2 H+ + AMP + PPi", "inputs": [ { "node_id": "R-ALL-111584", "reactome_id": "R-ALL-111584", "label": "XMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57464", "formula": "C10H11N4O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29472", "reactome_id": "R-ALL-29472", "label": "L-Gln", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58359", "formula": "C5H10N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113578", "reactome_id": "R-ALL-113578", "label": "GMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58115", "formula": "C10H12N5O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "GMP synthase (glutamine-hydrolyzing) activity of GMPS [cytosol]", "activity_id": "73785", "ec_numbers": [ "6.3.5.2" ], "accessions": null } ], "citations": [ { "id": "PMID:8089153", "title": "Human GMP synthetase. Protein purification, cloning, and functional expression of cDNA", "journal": "J Biol Chem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8089153/" }, { "id": "PMID:7706277", "title": "Biochemical characterization of human GMP synthetase", "journal": "J Biol Chem", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7706277/" } ], "x": 2660, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73794", "label": "IMP + H2O + NAD+ =\u003e XMP + NADH + H+ [IMPDH1,2]", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11709" ], "source_url": "https://reactome.org/content/detail/R-HSA-73794", "summary": "IMP + H2O + NAD+ =\u003e XMP + NADH + H+ [IMPDH1,2] is a Reactome release 97 curated reaction event. The source record connects H2O, IMP, NAD+ to H+, NADH, XMP in cytosol and cross-references RHEA:11709.", "equation": "H2O + IMP + NAD+ → H+ + NADH + XMP", "inputs": [ { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29602", "reactome_id": "R-ALL-29602", "label": "IMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58053", "formula": "C10H11N4O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111584", "reactome_id": "R-ALL-111584", "label": "XMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57464", "formula": "C10H11N4O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "IMP dehydrogenase activity of IMPDH tetramers [cytosol]", "activity_id": "73793", "ec_numbers": [ "1.1.1.205" ], "accessions": null } ], "citations": [ { "id": "PMID:7903306", "title": "Characterization of human type I and type II IMP dehydrogenases", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/7903306/" } ], "x": 2381.3333333333335, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73805", "label": "CAIR + Aspartate + ATP =\u003e SAICAR + ADP + Pi", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:22629" ], "source_url": "https://reactome.org/content/detail/R-HSA-73805", "summary": "CAIR + Aspartate + ATP =\u003e SAICAR + ADP + Pi is a Reactome release 97 curated reaction event. The source record connects CAIR, L-Asp, ATP to SAICAR, ADP, Pi in cytosol and cross-references RHEA:22629.", "equation": "CAIR + L-Asp + ATP → SAICAR + ADP + Pi + 2 H+", "inputs": [ { "node_id": "R-ALL-111395", "reactome_id": "R-ALL-111395", "label": "CAIR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:77657", "formula": "C9H12N3O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29448", "reactome_id": "R-ALL-29448", "label": "L-Asp", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29991", "formula": "C4H6NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111399", "reactome_id": "R-ALL-111399", "label": "SAICAR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58443", "formula": "C13H15N4O12P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoribosylaminoimidazolesuccinocarboxamide synthase activity of PAICS octamer [cytosol]", "activity_id": "73801", "ec_numbers": [ "6.3.2.6" ], "accessions": null } ], "citations": [ { "id": "PMID:2183217", "title": "Cloning of three human multifunctional de novo purine biosynthetic genes by functional complementation of yeast mutations", "journal": "Proc Natl Acad Sci USA", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2183217/" }, { "id": "PMID:17224163", "title": "Octameric structure of the human bifunctional enzyme PAICS in purine biosynthesis", "journal": "J Mol Biol", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17224163/" }, { "id": "PMID:18388293", "title": "Reversible compartmentalization of de novo purine biosynthetic complexes in living cells", "journal": "Science", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18388293/" } ], "x": 2660, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73810", "label": "FGAM + ATP =\u003e AIR + ADP + Pi", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23033" ], "source_url": "https://reactome.org/content/detail/R-HSA-73810", "summary": "FGAM + ATP =\u003e AIR + ADP + Pi is a Reactome release 97 curated reaction event. The source record connects FGAM, ATP to AIR, ADP, Pi in cytosol and cross-references RHEA:23033.", "equation": "FGAM + ATP → AIR + ADP + Pi + H+", "inputs": [ { "node_id": "R-ALL-111369", "reactome_id": "R-ALL-111369", "label": "FGAM", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:147287", "formula": "C8H15N3O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111370", "reactome_id": "R-ALL-111370", "label": "AIR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:137981", "formula": "C8H13N3O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoribosylformylglycinamidine cyclo-ligase activity of GART [cytosol]", "activity_id": "73809", "ec_numbers": [ "6.3.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:2147474", "title": "De novo purine nucleotide biosynthesis: cloning of human and avian cDNAs encoding the trifunctional glycinamide ribonucleotide synthetase-aminoimidazole ribonucleotide synthetase-glycinamide ribonucleotide transformylase by functional complementation in E. coli", "journal": "Nucleic Acids Res", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2147474/" }, { "id": "PMID:11381136", "title": "Localization of GAR transformylase in Escherichia coli and mammalian cells", "journal": "Proc Natl Acad Sci USA", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11381136/" }, { "id": "PMID:18388293", "title": "Reversible compartmentalization of de novo purine biosynthetic complexes in living cells", "journal": "Science", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18388293/" } ], "x": 2660, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73812", "label": "FGAR + L-Glutamine + ATP + H2O =\u003e FGAM + L-Glutamate + ADP + Pi", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17130" ], "source_url": "https://reactome.org/content/detail/R-HSA-73812", "summary": "FGAR + L-Glutamine + ATP + H2O =\u003e FGAM + L-Glutamate + ADP + Pi is a Reactome release 97 curated reaction event. The source record connects N-Formyl-GAR, L-Gln, ATP to FGAM, L-Glu, ADP in cytosol and cross-references RHEA:17130.", "equation": "N-Formyl-GAR + L-Gln + ATP + H2O → FGAM + L-Glu + ADP + Pi + H+", "inputs": [ { "node_id": "R-ALL-111354", "reactome_id": "R-ALL-111354", "label": "N-Formyl-GAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:147286", "formula": "C8H13N2O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29472", "reactome_id": "R-ALL-29472", "label": "L-Gln", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58359", "formula": "C5H10N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111369", "reactome_id": "R-ALL-111369", "label": "FGAM", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:147287", "formula": "C8H15N3O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoribosylformylglycinamidine synthase activity of PFAS [cytosol]", "activity_id": "73811", "ec_numbers": [ "6.3.5.3" ], "accessions": null } ], "citations": [ { "id": "PMID:8110788", "title": "Purification of, generation of monoclonal antibodies to, and mapping of phosphoribosyl N-formylglycinamide amidotransferase", "journal": "Biochemistry", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8110788/" }, { "id": "PMID:18388293", "title": "Reversible compartmentalization of de novo purine biosynthetic complexes in living cells", "journal": "Science", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18388293/" } ], "x": 2938.666666666667, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73814", "label": "5-Phosphoribosylamine + Glycine + ATP =\u003e GAR + ADP + Pi", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:17454" ], "source_url": "https://reactome.org/content/detail/R-HSA-73814", "summary": "5-Phosphoribosylamine + Glycine + ATP =\u003e GAR + ADP + Pi is a Reactome release 97 curated reaction event. The source record connects PRA, Gly, ATP to PRGA, ADP, Pi in cytosol and cross-references RHEA:17454.", "equation": "PRA + Gly + ATP → PRGA + ADP + Pi + H+", "inputs": [ { "node_id": "R-ALL-111292", "reactome_id": "R-ALL-111292", "label": "PRA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58681", "formula": "C5H11NO7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29424", "reactome_id": "R-ALL-29424", "label": "Gly", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57305", "formula": "C2H5NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111338", "reactome_id": "R-ALL-111338", "label": "PRGA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:143788", "formula": "C7H14N2O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoribosylamine-glycine ligase activity of GART [cytosol]", "activity_id": "73807", "ec_numbers": [ "6.3.4.13" ], "accessions": null } ], "citations": [ { "id": "PMID:2147474", "title": "De novo purine nucleotide biosynthesis: cloning of human and avian cDNAs encoding the trifunctional glycinamide ribonucleotide synthetase-aminoimidazole ribonucleotide synthetase-glycinamide ribonucleotide transformylase by functional complementation in E. coli", "journal": "Nucleic Acids Res", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2147474/" }, { "id": "PMID:11381136", "title": "Localization of GAR transformylase in Escherichia coli and mammalian cells", "journal": "Proc Natl Acad Sci USA", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11381136/" }, { "id": "PMID:18388293", "title": "Reversible compartmentalization of de novo purine biosynthetic complexes in living cells", "journal": "Science", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18388293/" } ], "x": 2938.666666666667, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73815", "label": "5-phospho-alpha-D-ribose 1-diphosphate (PRPP) + H2O + L-glutamine 5-phosphoribosylamine + L-glutamate +pyrophosphate", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-biosynthesis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14907" ], "source_url": "https://reactome.org/content/detail/R-HSA-73815", "summary": "5-phospho-alpha-D-ribose 1-diphosphate (PRPP) + H2O + L-glutamine 5-phosphoribosylamine + L-glutamate +pyrophosphate is a Reactome release 97 curated reaction event. The source record connects PRPP, L-Gln, H2O to PRA, L-Glu, PPi in cytosol and cross-references RHEA:14907.", "equation": "PRPP + L-Gln + H2O → PRA + L-Glu + PPi", "inputs": [ { "node_id": "R-ALL-73566", "reactome_id": "R-ALL-73566", "label": "PRPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58017", "formula": "C5H8O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29472", "reactome_id": "R-ALL-29472", "label": "L-Gln", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58359", "formula": "C5H10N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-111292", "reactome_id": "R-ALL-111292", "label": "PRA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58681", "formula": "C5H11NO7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "amidophosphoribosyltransferase activity of PPAT dimer [cytosol]", "activity_id": "73791", "ec_numbers": [ "2.4.2.14" ], "accessions": null } ], "citations": [ { "id": "PMID:4348202", "title": "Human glutamine phosphoribosylpyrophosphate amidotransferase. Kinetic and regulatory properties", "journal": "J Biol Chem", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4348202/" }, { "id": "PMID:4726295", "title": "Human glutamine phosphoribosylpyrophosphate amidotransferase. Two molecular forms interconvertible by purine ribonucleotides and phosphoribosylpyrophosphate", "journal": "J Biol Chem", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4726295/" }, { "id": "PMID:762062", "title": "Human amidophosphoribosyltransferase. An oxygen-sensitive iron-sulfur protein", "journal": "J Biol Chem", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/762062/" }, { "id": "PMID:8380692", "title": "Molecular cloning of human amidophosphoribosyltransferase", "journal": "Biochem Biophys Res Commun", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8380692/" }, { "id": "PMID:18388293", "title": "Reversible compartmentalization of de novo purine biosynthetic complexes in living cells", "journal": "Science", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18388293/" } ], "x": 2938.666666666667, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73912", "label": "acetoacetic acid + NADH + H+ beta-hydroxybutyrate + NAD+", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:20523" ], "source_url": "https://reactome.org/content/detail/R-HSA-73912", "summary": "acetoacetic acid + NADH + H+ beta-hydroxybutyrate + NAD+ is a Reactome release 97 curated reaction event. The source record connects ACA, NADH, H+ to bHBA, NAD+ in mitochondrial matrix and cross-references RHEA:20523.", "equation": "ACA + NADH + H+ → bHBA + NAD+", "inputs": [ { "node_id": "R-ALL-73909", "reactome_id": "R-ALL-73909", "label": "ACA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15344", "formula": "C4H6O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-73911", "reactome_id": "R-ALL-73911", "label": "bHBA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17066", "formula": "C4H8O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "3-hydroxybutyrate dehydrogenase activity of BDH1 tetramer [mitochondrial matrix]", "activity_id": "74155", "ec_numbers": [ "1.1.1.30" ], "accessions": null } ], "citations": [ { "id": "PMID:1639787", "title": "Molecular cloning and characterization of (R)-3-hydroxybutyrate dehydrogenase from human heart", "journal": "J Biol Chem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1639787/" } ], "x": 7461.333333333333, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73916", "label": "2 acetyl-CoA acetoacetyl-CoA + CoA", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:21037" ], "source_url": "https://reactome.org/content/detail/R-HSA-73916", "summary": "2 acetyl-CoA acetoacetyl-CoA + CoA is a Reactome release 97 curated reaction event. The source record connects Ac-CoA to ACA-CoA, CoA-SH in mitochondrial matrix and cross-references RHEA:21037.", "equation": "2 Ac-CoA → ACA-CoA + CoA-SH", "inputs": [ { "node_id": "R-ALL-113559", "reactome_id": "R-ALL-113559", "label": "Ac-CoA", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-73871", "reactome_id": "R-ALL-73871", "label": "ACA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57286", "formula": "C25H36N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acetyl-CoA C-acetyltransferase activity of ACAT1 tetramer [mitochondrial matrix]", "activity_id": "70842", "ec_numbers": [ "2.3.1.9" ], "accessions": null } ], "citations": [ { "id": "PMID:3709573", "title": "3-Ketothiolase deficiency", "journal": "Eur J Pediatr", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3709573/" } ], "x": 8018.666666666667, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73918", "label": "acetoacetyl-CoA+acetyl-CoA =\u003e HMG-CoA + CoASH", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:10189" ], "source_url": "https://reactome.org/content/detail/R-HSA-73918", "summary": "acetoacetyl-CoA+acetyl-CoA =\u003e HMG-CoA + CoASH is a Reactome release 97 curated reaction event. The source record connects ACA-CoA, Ac-CoA to HMG CoA, CoA-SH in mitochondrial matrix and cross-references RHEA:10189.", "equation": "ACA-CoA + Ac-CoA → HMG CoA + CoA-SH", "inputs": [ { "node_id": "R-ALL-73871", "reactome_id": "R-ALL-73871", "label": "ACA-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57286", "formula": "C25H36N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113559", "reactome_id": "R-ALL-113559", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-73917", "reactome_id": "R-ALL-73917", "label": "HMG CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15467", "formula": "C27H44N7O20P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "hydroxymethylglutaryl-CoA synthase activity of HMGCS2 [mitochondrial matrix]", "activity_id": "74170", "ec_numbers": [ "2.3.3.10" ], "accessions": null } ], "citations": [ { "id": "PMID:11479731", "title": "Genetic basis of mitochondrial HMG-CoA synthase deficiency", "journal": "Hum Genet", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11479731/" } ], "x": 8018.666666666667, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-73920", "label": "D-beta hydroxybutyrate+NAD+ acetoacetate+NADH+H+", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:20522" ], "source_url": "https://reactome.org/content/detail/R-HSA-73920", "summary": "D-beta hydroxybutyrate+NAD+ acetoacetate+NADH+H+ is a Reactome release 97 curated reaction event. The source record connects bHBA, NAD+ to ACA, NADH, H+ in mitochondrial matrix and cross-references RHEA:20522.", "equation": "bHBA + NAD+ → ACA + NADH + H+", "inputs": [ { "node_id": "R-ALL-73911", "reactome_id": "R-ALL-73911", "label": "bHBA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17066", "formula": "C4H8O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-73909", "reactome_id": "R-ALL-73909", "label": "ACA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15344", "formula": "C4H6O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "3-hydroxybutyrate dehydrogenase activity of BDH1 tetramer [mitochondrial matrix]", "activity_id": "74155", "ec_numbers": [ "1.1.1.30" ], "accessions": null } ], "citations": [ { "id": "PMID:1639787", "title": "Molecular cloning and characterization of (R)-3-hydroxybutyrate dehydrogenase from human heart", "journal": "J Biol Chem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1639787/" } ], "x": 7740, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-741449", "label": "SLC35B2,3 exchange cytosolic PAPS with Golgi PAP", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "Golgi membrane · Golgi lumen · cytosol", "compartment_ids": [ "GO:0000139", "GO:0005796", "GO:0005829" ], "rhea_ids": [ "RHEA:76065", "RHEA:76063" ], "source_url": "https://reactome.org/content/detail/R-HSA-741449", "summary": "SLC35B2,3 exchange cytosolic PAPS with Golgi PAP is a Reactome release 97 curated reaction event. The source record connects PAPS, PAP to PAPS, PAP in Golgi membrane · Golgi lumen · cytosol and cross-references RHEA:76065, RHEA:76063.", "equation": "PAPS + PAP → PAPS + PAP", "inputs": [ { "node_id": "R-ALL-158471", "reactome_id": "R-ALL-158471", "label": "PAPS", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58339", "formula": "C10H11N5O13P2S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-742343", "reactome_id": "R-ALL-742343", "label": "PAP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58343", "formula": "C10H11N5O10P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-741440", "reactome_id": "R-ALL-741440", "label": "PAPS", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58339", "formula": "C10H11N5O13P2S", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-158466", "reactome_id": "R-ALL-158466", "label": "PAP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58343", "formula": "C10H11N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "3'-phosphoadenosine 5'-phosphosulfate transmembrane transporter activity of SLC35B2,3 [Golgi membrane]", "activity_id": "741439", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:8619989", "title": "Identification and partial purification of PAPS translocase", "journal": "Biochemistry", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8619989/" }, { "id": "PMID:12716889", "title": "Molecular cloning and identification of 3'-phosphoadenosine 5'-phosphosulfate transporter", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12716889/" }, { "id": "PMID:16492677", "title": "Molecular cloning and characterization of a novel 3'-phosphoadenosine 5'-phosphosulfate transporter, PAPST2", "journal": "J Biol Chem", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16492677/" }, { "id": "PMID:35325049", "title": "Biallelic variants in SLC35B2 cause a novel chondrodysplasia with hypomyelinating leukodystrophy", "journal": "Brain", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/35325049/" } ], "x": 2381.3333333333335, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-74177", "label": "OXCT dimers transfer CoA from SUCC-CoA to ACA, forming ACA-CoA", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:25481" ], "source_url": "https://reactome.org/content/detail/R-HSA-74177", "summary": "OXCT dimers transfer CoA from SUCC-CoA to ACA, forming ACA-CoA is a Reactome release 97 curated reaction event. The source record connects ACA, SUCC-CoA to ACA-CoA, SUCCA in mitochondrial matrix and cross-references RHEA:25481.", "equation": "ACA + SUCC-CoA → ACA-CoA + SUCCA", "inputs": [ { "node_id": "R-ALL-73909", "reactome_id": "R-ALL-73909", "label": "ACA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15344", "formula": "C4H6O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70958", "reactome_id": "R-ALL-70958", "label": "SUCC-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57292", "formula": "C25H35N7O19P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-73871", "reactome_id": "R-ALL-73871", "label": "ACA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57286", "formula": "C25H36N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113536", "reactome_id": "R-ALL-113536", "label": "SUCCA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30031", "formula": "C4H4O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "succinyl-CoA:3-oxo-acid CoA-transferase activity of OXCT dimers [mitochondrial matrix]", "activity_id": "74278", "ec_numbers": [ "2.8.3.5" ], "accessions": null } ], "citations": [ { "id": "PMID:8751852", "title": "Succinyl CoA: 3-oxoacid CoA transferase (SCOT): human cDNA cloning, human chromosomal mapping to 5p13, and mutation detection in a SCOT-deficient patient", "journal": "Am J Hum Genet", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8751852/" }, { "id": "PMID:11756565", "title": "Cloning and characterization of a human orthologue of testis-specific succinyl CoA: 3-oxo acid CoA transferase (Scot-t) cDNA", "journal": "Mol. Hum. Reprod.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11756565/" } ], "x": 7461.333333333333, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-74180", "label": "HMG CoA =\u003e acetoacetic acid + acetyl CoA", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:24405" ], "source_url": "https://reactome.org/content/detail/R-HSA-74180", "summary": "HMG CoA =\u003e acetoacetic acid + acetyl CoA is a Reactome release 97 curated reaction event. The source record connects HMG CoA to Ac-CoA, ACA in mitochondrial matrix and cross-references RHEA:24405.", "equation": "HMG CoA → Ac-CoA + ACA", "inputs": [ { "node_id": "R-ALL-73917", "reactome_id": "R-ALL-73917", "label": "HMG CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15467", "formula": "C27H44N7O20P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-113559", "reactome_id": "R-ALL-113559", "label": "Ac-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73909", "reactome_id": "R-ALL-73909", "label": "ACA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15344", "formula": "C4H6O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "hydroxymethylglutaryl-CoA lyase activity of HMGCL dimer [mitochondrial matrix]", "activity_id": "74179", "ec_numbers": [ "4.1.3.4" ], "accessions": null } ], "citations": [ { "id": "PMID:8440722", "title": "3-Hydroxy-3-methylglutaryl coenzyme A lyase (HL). Cloning of human and chicken liver HL cDNAs and characterization of a mutation causing human HL deficiency.", "journal": "J Biol Chem", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/8440722/" } ], "x": 7461.333333333333, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-74207", "label": "dA, dG, or dI + ATP =\u003e dAMP, dGMP, or dIMP + ADP (DGUOK)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:23453", "RHEA:19202" ], "source_url": "https://reactome.org/content/detail/R-HSA-74207", "summary": "dA, dG, or dI + ATP =\u003e dAMP, dGMP, or dIMP + ADP (DGUOK) is a Reactome release 97 curated reaction event. The source record connects dA, dG, dI, ATP to dAMP, dGMP, dIMP, ADP, H+ in mitochondrial matrix and cross-references RHEA:23453, RHEA:19202.", "equation": "dA, dG, dI + ATP → dAMP, dGMP, dIMP + ADP + H+", "inputs": [ { "node_id": "R-ALL-500224", "reactome_id": "R-ALL-500224", "label": "dA, dG, dI", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500222", "reactome_id": "R-ALL-500222", "label": "dAMP, dGMP, dIMP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "deoxynucleoside kinase activity of DGUOK dimer [mitochondrial matrix]", "activity_id": "74206", "ec_numbers": [ "2.7.1.145" ], "accessions": null } ], "citations": [ { "id": "PMID:8692979", "title": "Cloning and expression of human deoxyguanosine kinase cDNA", "journal": "Proc Natl Acad Sci USA", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8692979/" }, { "id": "PMID:2845866", "title": "Properties of a highly purified mitochondrial deoxyguanosine kinase", "journal": "Arch Biochem Biophys", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/2845866/" }, { "id": "PMID:11427893", "title": "Structural basis for substrate specificities of cellular deoxyribonucleoside kinases", "journal": "Nat Struct Biol", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11427893/" } ], "x": 3878.666666666667, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-74213", "label": "APRT catalyzes the conversion of adenine to AMP", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:16611" ], "source_url": "https://reactome.org/content/detail/R-HSA-74213", "summary": "APRT catalyzes the conversion of adenine to AMP is a Reactome release 97 curated reaction event. The source record connects Ade, PRPP to AMP, PPi in cytosol and cross-references RHEA:16611.", "equation": "Ade + PRPP → AMP + PPi", "inputs": [ { "node_id": "R-ALL-83951", "reactome_id": "R-ALL-83951", "label": "Ade", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16708", "formula": "C5H5N5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73566", "reactome_id": "R-ALL-73566", "label": "PRPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58017", "formula": "C5H8O14P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111294", "reactome_id": "R-ALL-111294", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "adenine phosphoribosyltransferase activity of APRT dimer [cytosol]", "activity_id": "74212", "ec_numbers": [ "2.4.2.7" ], "accessions": null } ], "citations": [ { "id": "PMID:457664", "title": "Human adenine phosphoribosyltransferase. Affinity purification, subunit structure, amino acid composition, and peptide mapping.", "journal": "J Biol Chem", "year": 1979, "url": "https://pubmed.ncbi.nlm.nih.gov/457664/" }, { "id": "PMID:18399692", "title": "Structural complexes of human adenine phosphoribosyltransferase reveal novel features of the APRT catalytic mechanism", "journal": "J Biomol Struct Dyn", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18399692/" } ], "x": 3600, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-74241", "label": "ADA catalyzes the deamination of (deoxy)adenosine", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:28191", "RHEA:24409" ], "source_url": "https://reactome.org/content/detail/R-HSA-74241", "summary": "ADA catalyzes the deamination of (deoxy)adenosine is a Reactome release 97 curated reaction event. The source record connects Ade-Rib, dA, H2O, H+ to Ino, dI, NH4+ in cytosol and cross-references RHEA:28191, RHEA:24409.", "equation": "Ade-Rib, dA + H2O + H+ → Ino, dI + NH4+", "inputs": [ { "node_id": "R-ALL-500173", "reactome_id": "R-ALL-500173", "label": "Ade-Rib, dA", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-500172", "reactome_id": "R-ALL-500172", "label": "Ino, dI", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "deaminase activity of ADA [cytosol]", "activity_id": "74234", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:3182793", "title": "Mutant human adenosine deaminase alleles and their expression by transfection into fibroblasts", "journal": "J Biol Chem", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3182793/" } ], "x": 3321.3333333333335, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-744231", "label": "SLC35D2 exchanges UDP-GlcNAc for UMP", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "Golgi membrane", "compartment_ids": [ "GO:0000139" ], "rhea_ids": [ "RHEA:72695" ], "source_url": "https://reactome.org/content/detail/R-HSA-744231", "summary": "SLC35D2 exchanges UDP-GlcNAc for UMP is a Reactome release 97 curated reaction event. The source record connects UMP, UDP-GlcNAc to UMP, UDP-GlcNAc in Golgi membrane and cross-references RHEA:72695.", "equation": "UMP + UDP-GlcNAc → UMP + UDP-GlcNAc", "inputs": [ { "node_id": "R-ALL-735698", "reactome_id": "R-ALL-735698", "label": "UMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57865", "formula": "C9H11N2O9P", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-162756", "reactome_id": "R-ALL-162756", "label": "UDP-GlcNAc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57705", "formula": "C17H25N3O17P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-109402", "reactome_id": "R-ALL-109402", "label": "UMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57865", "formula": "C9H11N2O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-914003", "reactome_id": "R-ALL-914003", "label": "UDP-GlcNAc", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57705", "formula": "C17H25N3O17P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "nucleotide-sugar transmembrane transporter activity of SLC35D2 [Golgi membrane]", "activity_id": "744232", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:15082721", "title": "Molecular cloning and characterization of a human multisubstrate specific nucleotide-sugar transporter homologous to Drosophila fringe connection", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15082721/" }, { "id": "PMID:15607426", "title": "Identification and characterization of human Golgi nucleotide sugar transporter SLC35D2, a novel member of the SLC35 nucleotide sugar transporter family", "journal": "Genomics", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15607426/" } ], "x": 2660, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-75848", "label": "ACLY tetramer transforms CIT to Ac-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:21162" ], "source_url": "https://reactome.org/content/detail/R-HSA-75848", "summary": "ACLY tetramer transforms CIT to Ac-CoA is a Reactome release 97 curated reaction event. The source record connects CIT, CoA-SH, ATP to Ac-CoA, ADP, OA in cytosol and cross-references RHEA:21162.", "equation": "CIT + CoA-SH + ATP → Ac-CoA + ADP + OA + Pi", "inputs": [ { "node_id": "R-ALL-76190", "reactome_id": "R-ALL-76190", "label": "CIT", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16947", "formula": "C6H5O7", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76213", "reactome_id": "R-ALL-76213", "label": "OA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "ATP citrate synthase activity of ACLY tetramer [cytosol]", "activity_id": "76189", "ec_numbers": [ "2.3.3.8" ], "accessions": null } ], "citations": [ { "id": "PMID:1371749", "title": "Cloning and expression of a human ATP-citrate lyase cDNA", "journal": "Eur J Biochem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1371749/" }, { "id": "PMID:23932781", "title": "Acetylation stabilizes ATP-citrate lyase to promote lipid biosynthesis and tumor growth", "journal": "Mol. Cell", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23932781/" }, { "id": "PMID:24286864", "title": "Cytosolic functions of MORC2 in lipogenesis and adipogenesis", "journal": "Biochim. Biophys. Acta", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24286864/" }, { "id": "PMID:37051670", "title": "Determiners of cell fates: the tricarboxylic acid cycle versus the citrate-malate shuttle", "journal": "Protein Cell", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/37051670/" }, { "id": "PMID:36581208", "title": "Regulation and function of the mammalian tricarboxylic acid cycle", "journal": "J Biol Chem", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/36581208/" }, { "id": "PMID:33917812", "title": "Acetyl-CoA Metabolism and Histone Acetylation in the Regulation of Aging and Lifespan", "journal": "Antioxidants (Basel)", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/33917812/" } ], "x": 590, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-75851", "label": "Btn-ACACA:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11309" ], "source_url": "https://reactome.org/content/detail/R-HSA-75851", "summary": "Btn-ACACA:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoA is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, ATP, HCO3- to ADP, Pi, Mal-CoA in cytosol and cross-references RHEA:11309.", "equation": "Ac-CoA + ATP + HCO3- → ADP + Pi + Mal-CoA + H+", "inputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111627", "reactome_id": "R-ALL-111627", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29508", "reactome_id": "R-ALL-29508", "label": "Mal-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57384", "formula": "C24H33N7O19P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acetyl-CoA carboxylase activity of Btn-ACACA:2Mn2+ polymer [cytosol]", "activity_id": "76186", "ec_numbers": [ "6.4.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:17223360", "title": "Expression, purification, and characterization of human acetyl-CoA carboxylase 2", "journal": "Protein Expr Purif", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17223360/" }, { "id": "PMID:9099716", "title": "Human acetyl-CoA carboxylase 2. Molecular cloning, characterization, chromosomal mapping, and evidence for two isoforms.", "journal": "J Biol Chem", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9099716/" }, { "id": "PMID:10677481", "title": "The subcellular localization of acetyl-CoA carboxylase 2", "journal": "Proc Natl Acad Sci U S A", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10677481/" }, { "id": "PMID:20457939", "title": "Induced polymerization of mammalian acetyl-CoA carboxylase by MIG12 provides a tertiary level of regulation of fatty acid synthesis", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20457939/" }, { "id": "PMID:22123817", "title": "Selectivity in post-translational biotin addition to five human carboxylases", "journal": "J. Biol. Chem.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22123817/" }, { "id": "PMID:18556348", "title": "Hepatic expression of the SPOT 14 (S14) paralog S14-related (Mid1 interacting protein) is regulated by dietary carbohydrate", "journal": "Endocrinology", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18556348/" } ], "x": 868.6666666666667, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-75872", "label": "Conversion of malonyl-CoA and acetyl-CoA to palmitate", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14994" ], "source_url": "https://reactome.org/content/detail/R-HSA-75872", "summary": "Conversion of malonyl-CoA and acetyl-CoA to palmitate is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, Mal-CoA, NADPH to PALM, CO2, NADP+ in cytosol and cross-references RHEA:14994.", "equation": "Ac-CoA + 7 Mal-CoA + 14 NADPH + 14 H+ → PALM + 7 CO2 + 14 NADP+ + 8 CoA-SH + 6 H2O", "inputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29508", "reactome_id": "R-ALL-29508", "label": "Mal-CoA", "role": "input", "stoichiometry": 7, "chebi_id": "CHEBI:57384", "formula": "C24H33N7O19P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 14, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 14, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-3632881", "reactome_id": "R-ALL-3632881", "label": "PALM", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:7896", "formula": "C16H31O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 7, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 14, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 6, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "fatty acid synthase activity of FASN dimer [cytosol]", "activity_id": "539119", "ec_numbers": [ "2.3.1.85" ], "accessions": null } ], "citations": [ { "id": "PMID:7567999", "title": "Human fatty acid synthase: properties and molecular cloning", "journal": "Proc Natl Acad Sci U S A", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7567999/" }, { "id": "PMID:12689621", "title": "Structural and functional organization of the animal fatty acid synthase.", "journal": "Prog Lipid Res", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12689621/" } ], "x": 1251.3333333333333, "y": 1727.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-75887", "label": "Conversion of Glycerol to Glycerol-3-phosphate", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:21645" ], "source_url": "https://reactome.org/content/detail/R-HSA-75887", "summary": "Conversion of Glycerol to Glycerol-3-phosphate is a Reactome release 97 curated reaction event. The source record connects Glycerol, ATP to G3P, ADP in cytosol and cross-references RHEA:21645.", "equation": "Glycerol + ATP → G3P + ADP", "inputs": [ { "node_id": "R-ALL-76116", "reactome_id": "R-ALL-76116", "label": "Glycerol", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17754", "formula": "C3H8O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76114", "reactome_id": "R-ALL-76114", "label": "G3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57597", "formula": "C3H7O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycerol kinase activity of GK,GK2,GK3P [cytosol]", "activity_id": "76115", "ec_numbers": [ "2.7.1.30" ], "accessions": null } ], "citations": [ { "id": "PMID:15845384", "title": "Human and murine glycerol kinase: influence of exon 18 alternative splicing on function", "journal": "Biochem Biophys Res Commun", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15845384/" } ], "x": 3321.3333333333335, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-75889", "label": "DHAP is converted to G3P by GPD1/GPD1L", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:11094" ], "source_url": "https://reactome.org/content/detail/R-HSA-75889", "summary": "DHAP is converted to G3P by GPD1/GPD1L is a Reactome release 97 curated reaction event. The source record connects DHAP, NADH, H+ to G3P, NAD+ in cytosol and cross-references RHEA:11094.", "equation": "DHAP + NADH + H+ → G3P + NAD+", "inputs": [ { "node_id": "R-ALL-75970", "reactome_id": "R-ALL-75970", "label": "DHAP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57642", "formula": "C3H5O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76114", "reactome_id": "R-ALL-76114", "label": "G3P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57597", "formula": "C3H7O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glycerol-3-phosphate dehydrogenase [NAD(P)+] activity of GPD1/GPD1L homodimer [cytosol]", "activity_id": "1500607", "ec_numbers": [ "1.1.1.94" ], "accessions": null } ], "citations": [ { "id": "PMID:19666841", "title": "GPD1L links redox state to cardiac excitability by PKC-dependent phosphorylation of the sodium channel SCN5A", "journal": "Am J Physiol Heart Circ Physiol", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19666841/" }, { "id": "PMID:16460752", "title": "Crystal structures of human glycerol 3-phosphate dehydrogenase 1 (GPD1)", "journal": "J Mol Biol", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16460752/" } ], "x": 2660, "y": 1678.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-76496", "label": "PTGIS, CYP8A1 isomerise PGH2 to PGI2", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "arachidonate", "biological-oxidations", "fatty-acid-metabolism", "phase-one" ], "compartment": "endoplasmic reticulum membrane · endoplasmic reticulum lumen", "compartment_ids": [ "GO:0005789", "GO:0005788" ], "rhea_ids": [ "RHEA:23581" ], "source_url": "https://reactome.org/content/detail/R-HSA-76496", "summary": "PTGIS, CYP8A1 isomerise PGH2 to PGI2 is a Reactome release 97 curated reaction event. The source record connects PGH2 to PGI2 in endoplasmic reticulum membrane · endoplasmic reticulum lumen and cross-references RHEA:23581.", "equation": "PGH2 → PGI2", "inputs": [ { "node_id": "R-ALL-30138", "reactome_id": "R-ALL-30138", "label": "PGH2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57405", "formula": "C20H31O5", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-31593", "reactome_id": "R-ALL-31593", "label": "PGI2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57403", "formula": "C20H31O5", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "prostaglandin-I synthase activity of PTGIS,CYP8B1 [endoplasmic reticulum membrane]", "activity_id": "76495", "ec_numbers": [ "5.3.99.4" ], "accessions": null } ], "citations": [ { "id": "PMID:15115769", "title": "Purification and characterization of recombinant human prostacyclin synthase", "journal": "J Biochem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15115769/" }, { "id": "PMID:17641676", "title": "1-Methylnicotinamide (MNA), a primary metabolite of nicotinamide, exerts anti-thrombotic activity mediated by a cyclooxygenase-2/prostacyclin pathway", "journal": "Br. J. Pharmacol.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17641676/" } ], "x": 6800, "y": 3379.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-76500", "label": "TBXAS1 isomerises PGH2 to TXA2", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "arachidonate", "biological-oxidations", "fatty-acid-metabolism", "phase-one" ], "compartment": "endoplasmic reticulum membrane · endoplasmic reticulum lumen", "compartment_ids": [ "GO:0005789", "GO:0005788" ], "rhea_ids": [ "RHEA:17138" ], "source_url": "https://reactome.org/content/detail/R-HSA-76500", "summary": "TBXAS1 isomerises PGH2 to TXA2 is a Reactome release 97 curated reaction event. The source record connects PGH2 to TXA2 in endoplasmic reticulum membrane · endoplasmic reticulum lumen and cross-references RHEA:17138.", "equation": "PGH2 → TXA2", "inputs": [ { "node_id": "R-ALL-30138", "reactome_id": "R-ALL-30138", "label": "PGH2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57405", "formula": "C20H31O5", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-32879", "reactome_id": "R-ALL-32879", "label": "TXA2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57445", "formula": "C20H31O5", "compartment": "endoplasmic reticulum lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "thromboxane-A synthase activity of TBXAS1 [endoplasmic reticulum membrane]", "activity_id": "76499", "ec_numbers": [ "5.3.99.5" ], "accessions": null } ], "citations": [ { "id": "PMID:7925341", "title": "Characterization of the human gene (TBXAS1) encoding thromboxane synthase", "journal": "Eur J Biochem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7925341/" }, { "id": "PMID:11465543", "title": "Identification of genetic variants in the human thromboxane synthase gene (CYP5A1)", "journal": "Mutat Res", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11465543/" } ], "x": 7078.666666666667, "y": 3379.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-76590", "label": "AMP + H2O =\u003e IMP + NH4+ (AMPD)", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-salvage" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14778" ], "source_url": "https://reactome.org/content/detail/R-HSA-76590", "summary": "AMP + H2O =\u003e IMP + NH4+ (AMPD) is a Reactome release 97 curated reaction event. The source record connects AMP, H2O, H+ to NH4+, IMP in cytosol and cross-references RHEA:14778.", "equation": "AMP + H2O + H+ → NH4+ + IMP", "inputs": [ { "node_id": "R-ALL-76577", "reactome_id": "R-ALL-76577", "label": "AMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29602", "reactome_id": "R-ALL-29602", "label": "IMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58053", "formula": "C10H11N4O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "AMP deaminase activity of AMPD tetramers [cytosol]", "activity_id": "76587", "ec_numbers": [ "3.5.4.6" ], "accessions": null } ], "citations": [ { "id": "PMID:1429593", "title": "Molecular cloning of AMP deaminase isoform L. Sequence and bacterial expression of human AMPD2 cDNA", "journal": "J Biol Chem", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1429593/" }, { "id": "PMID:9857047", "title": "Novel aspects of tetramer assembly and N-terminal domain structure and function are revealed by recombinant expression of human AMP deaminase isoforms", "journal": "J Biol Chem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9857047/" } ], "x": 3878.666666666667, "y": 3046.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77254", "label": "(S)-3-Hydroxydodecanoyl-CoA+NAD =\u003e 3-Oxododecanoyl-CoA+NADH+H", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31180" ], "source_url": "https://reactome.org/content/detail/R-HSA-77254", "summary": "(S)-3-Hydroxydodecanoyl-CoA+NAD =\u003e 3-Oxododecanoyl-CoA+NADH+H is a Reactome release 97 curated reaction event. The source record connects NAD+, (S)-3-Hydroxydodecanoyl-CoA to NADH, H+, 3-Oxododecanoyl-CoA in mitochondrial matrix and cross-references RHEA:31180.", "equation": "NAD+ + (S)-3-Hydroxydodecanoyl-CoA → NADH + H+ + 3-Oxododecanoyl-CoA", "inputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77252", "reactome_id": "R-ALL-77252", "label": "(S)-3-Hydroxydodecanoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62558", "formula": "C33H54N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77253", "reactome_id": "R-ALL-77253", "label": "3-Oxododecanoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62615", "formula": "C33H52N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of HADH dimer [mitochondrial matrix]", "activity_id": "71061", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:8687463", "title": "Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence.", "journal": "Biochem Biophys Res Commun", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8687463/" } ], "x": 1251.3333333333333, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77263", "label": "lauroyl-CoA+FAD =\u003e 2-trans-Dodecenoyl-CoA+FADH2", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31040" ], "source_url": "https://reactome.org/content/detail/R-HSA-77263", "summary": "lauroyl-CoA+FAD =\u003e 2-trans-Dodecenoyl-CoA+FADH2 is a Reactome release 97 curated reaction event. The source record connects FAD, LAU-CoA, H+ to FADH2, 2-trans-Dodecenoyl-CoA in mitochondrial matrix and cross-references RHEA:31040.", "equation": "FAD + LAU-CoA + H+ → FADH2 + 2-trans-Dodecenoyl-CoA", "inputs": [ { "node_id": "R-ALL-113596", "reactome_id": "R-ALL-113596", "label": "FAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57692", "formula": "C27H30N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77261", "reactome_id": "R-ALL-77261", "label": "LAU-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57375", "formula": "C33H54N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31649", "reactome_id": "R-ALL-31649", "label": "FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58307", "formula": "C27H33N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77255", "reactome_id": "R-ALL-77255", "label": "2-trans-Dodecenoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57330", "formula": "C33H52N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acyl-CoA dehydrogenase activity of ACADL tetramer [mitochondrial matrix]", "activity_id": "77259", "ec_numbers": [ "1.3.99.3" ], "accessions": null } ], "citations": [ { "id": "PMID:13295225", "title": "On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. II. The electron-transferring flavoprotein.", "journal": "J Biol Chem", "year": 1956, "url": "https://pubmed.ncbi.nlm.nih.gov/13295225/" }, { "id": "PMID:1540149", "title": "Beta-oxidation of long-chain fatty acids by human fibroblasts: evidence for a novel long-chain acyl-coenzyme A dehydrogenase.", "journal": "Biochem Biophys Res Commun", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1540149/" } ], "x": 1530, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77274", "label": "myristoyl-CoA+FAD =\u003e trans-tetradec-2-enoyl-CoA+FADH2", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:28359", "RHEA:47317" ], "source_url": "https://reactome.org/content/detail/R-HSA-77274", "summary": "myristoyl-CoA+FAD =\u003e trans-tetradec-2-enoyl-CoA+FADH2 is a Reactome release 97 curated reaction event. The source record connects FAD, MYS-CoA, H+ to FADH2, trans-Tetradec-2-enoyl-CoA in mitochondrial matrix and cross-references RHEA:28359, RHEA:47317.", "equation": "FAD + MYS-CoA + H+ → FADH2 + trans-Tetradec-2-enoyl-CoA", "inputs": [ { "node_id": "R-ALL-113596", "reactome_id": "R-ALL-113596", "label": "FAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57692", "formula": "C27H30N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77272", "reactome_id": "R-ALL-77272", "label": "MYS-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57385", "formula": "C35H58N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31649", "reactome_id": "R-ALL-31649", "label": "FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58307", "formula": "C27H33N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77273", "reactome_id": "R-ALL-77273", "label": "trans-Tetradec-2-enoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:61405", "formula": "C35H56N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acyl-CoA dehydrogenase activity of ACADL tetramer [mitochondrial matrix]", "activity_id": "77259", "ec_numbers": [ "1.3.99.3" ], "accessions": null } ], "citations": [ { "id": "PMID:13295225", "title": "On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. II. The electron-transferring flavoprotein.", "journal": "J Biol Chem", "year": 1956, "url": "https://pubmed.ncbi.nlm.nih.gov/13295225/" }, { "id": "PMID:1540149", "title": "Beta-oxidation of long-chain fatty acids by human fibroblasts: evidence for a novel long-chain acyl-coenzyme A dehydrogenase.", "journal": "Biochem Biophys Res Commun", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1540149/" } ], "x": 1808.6666666666667, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77283", "label": "(S)-3-Hydroxytetradecanoyl-CoA+NAD =\u003e 3-Oxotetradecanoyl-CoA+NADH+H", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31168" ], "source_url": "https://reactome.org/content/detail/R-HSA-77283", "summary": "(S)-3-Hydroxytetradecanoyl-CoA+NAD =\u003e 3-Oxotetradecanoyl-CoA+NADH+H is a Reactome release 97 curated reaction event. The source record connects NAD+, (S)-3-Hydroxytetradecanoyl-CoA to NADH, H+, 3-Oxotetradecanoyl-CoA in mitochondrial matrix and cross-references RHEA:31168.", "equation": "NAD+ + (S)-3-Hydroxytetradecanoyl-CoA → NADH + H+ + 3-Oxotetradecanoyl-CoA", "inputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77276", "reactome_id": "R-ALL-77276", "label": "(S)-3-Hydroxytetradecanoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62614", "formula": "C35H58N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77269", "reactome_id": "R-ALL-77269", "label": "3-Oxotetradecanoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62543", "formula": "C35H56N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of Trifunctional Protein [mitochondrial matrix]", "activity_id": "77282", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:1550553", "title": "Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria.", "journal": "Biochem Biophys Res Commun", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1550553/" } ], "x": 1251.3333333333333, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77299", "label": "palmitoyl-CoA+FAD =\u003e trans-Hexadec-2-enoyl-CoA+FADH2", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:30748", "RHEA:43449" ], "source_url": "https://reactome.org/content/detail/R-HSA-77299", "summary": "palmitoyl-CoA+FAD =\u003e trans-Hexadec-2-enoyl-CoA+FADH2 is a Reactome release 97 curated reaction event. The source record connects PalmCoA, FAD, H+ to FADH2, trans-Hexadec-2-enoyl-CoA in mitochondrial matrix and cross-references RHEA:30748, RHEA:43449.", "equation": "PalmCoA + FAD + H+ → FADH2 + trans-Hexadec-2-enoyl-CoA", "inputs": [ { "node_id": "R-ALL-77281", "reactome_id": "R-ALL-77281", "label": "PalmCoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57379", "formula": "C37H62N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113596", "reactome_id": "R-ALL-113596", "label": "FAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57692", "formula": "C27H30N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31649", "reactome_id": "R-ALL-31649", "label": "FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58307", "formula": "C27H33N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77284", "reactome_id": "R-ALL-77284", "label": "trans-Hexadec-2-enoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:61526", "formula": "C37H60N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acyl-CoA dehydrogenase activity of ACADVL dimer [mitochondrial matrix]", "activity_id": "77280", "ec_numbers": [ "1.3.99.3" ], "accessions": null } ], "citations": [ { "id": "PMID:13295225", "title": "On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. II. The electron-transferring flavoprotein.", "journal": "J Biol Chem", "year": 1956, "url": "https://pubmed.ncbi.nlm.nih.gov/13295225/" }, { "id": "PMID:1540149", "title": "Beta-oxidation of long-chain fatty acids by human fibroblasts: evidence for a novel long-chain acyl-coenzyme A dehydrogenase.", "journal": "Biochem Biophys Res Commun", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1540149/" } ], "x": 1530, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77303", "label": "(S)-3-Hydroxyhexadecanoyl-CoA+NAD =\u003e 3-Oxopalmitoyl-CoA+NADH+H", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31160" ], "source_url": "https://reactome.org/content/detail/R-HSA-77303", "summary": "(S)-3-Hydroxyhexadecanoyl-CoA+NAD =\u003e 3-Oxopalmitoyl-CoA+NADH+H is a Reactome release 97 curated reaction event. The source record connects (S)-3-Hydroxyhexadecanoyl-CoA, NAD+ to NADH, 3-Oxopalmitoyl-CoA, H+ in mitochondrial matrix and cross-references RHEA:31160.", "equation": "(S)-3-Hydroxyhexadecanoyl-CoA + NAD+ → NADH + 3-Oxopalmitoyl-CoA + H+", "inputs": [ { "node_id": "R-ALL-77300", "reactome_id": "R-ALL-77300", "label": "(S)-3-Hydroxyhexadecanoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62613", "formula": "C37H62N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77302", "reactome_id": "R-ALL-77302", "label": "3-Oxopalmitoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57349", "formula": "C37H60N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of Trifunctional Protein [mitochondrial matrix]", "activity_id": "77282", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:1550553", "title": "Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria.", "journal": "Biochem Biophys Res Commun", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1550553/" } ], "x": 1808.6666666666667, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77312", "label": "(S)-Hydroxybutanoyl-CoA+NAD =\u003e Acetoacetyl-CoA+NADH+H", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:30800" ], "source_url": "https://reactome.org/content/detail/R-HSA-77312", "summary": "(S)-Hydroxybutanoyl-CoA+NAD =\u003e Acetoacetyl-CoA+NADH+H is a Reactome release 97 curated reaction event. The source record connects NAD+, 3HB-CoA to NADH, H+, ACA-CoA in mitochondrial matrix and cross-references RHEA:30800.", "equation": "NAD+ + 3HB-CoA → NADH + H+ + ACA-CoA", "inputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77251", "reactome_id": "R-ALL-77251", "label": "3HB-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57316", "formula": "C25H38N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-73871", "reactome_id": "R-ALL-73871", "label": "ACA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57286", "formula": "C25H36N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of HADH dimer [mitochondrial matrix]", "activity_id": "71061", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:8687463", "title": "Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence.", "journal": "Biochem Biophys Res Commun", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8687463/" }, { "id": "PMID:10231530", "title": "Biochemical characterization and crystal structure determination of human heart short chain L-3-hydroxyacyl-CoA dehydrogenase provide insights into catalytic mechanism.", "journal": "Biochemistry", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10231530/" } ], "x": 1251.3333333333333, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77319", "label": "Butanoyl-CoA+FAD =\u003e Crotonoyl-CoA+FADH2", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:30732" ], "source_url": "https://reactome.org/content/detail/R-HSA-77319", "summary": "Butanoyl-CoA+FAD =\u003e Crotonoyl-CoA+FADH2 is a Reactome release 97 curated reaction event. The source record connects FAD, BT-CoA, H+ to FADH2, Crotonoyl-CoA in mitochondrial matrix and cross-references RHEA:30732.", "equation": "FAD + BT-CoA + H+ → FADH2 + Crotonoyl-CoA", "inputs": [ { "node_id": "R-ALL-113596", "reactome_id": "R-ALL-113596", "label": "FAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57692", "formula": "C27H30N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77318", "reactome_id": "R-ALL-77318", "label": "BT-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57371", "formula": "C25H38N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31649", "reactome_id": "R-ALL-31649", "label": "FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58307", "formula": "C27H33N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77313", "reactome_id": "R-ALL-77313", "label": "Crotonoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57332", "formula": "C25H36N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acyl-CoA dehydrogenase activity of ACADS tetramer [mitochondrial matrix]", "activity_id": "77317", "ec_numbers": [ "1.3.99.3" ], "accessions": null } ], "citations": [ { "id": "PMID:13295225", "title": "On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. II. The electron-transferring flavoprotein.", "journal": "J Biol Chem", "year": 1956, "url": "https://pubmed.ncbi.nlm.nih.gov/13295225/" }, { "id": "PMID:3597357", "title": "Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver.", "journal": "J Biol Chem", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3597357/" } ], "x": 1530, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77323", "label": "(S)-Hydroxyhexanoyl-CoA+NAD =\u003e 3-Oxohexanoyl-CoA+NADH+H", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31144" ], "source_url": "https://reactome.org/content/detail/R-HSA-77323", "summary": "(S)-Hydroxyhexanoyl-CoA+NAD =\u003e 3-Oxohexanoyl-CoA+NADH+H is a Reactome release 97 curated reaction event. The source record connects NAD+, (S)-Hydroxyhexanoyl-CoA to NADH, H+, 3-Oxohexanoyl-CoA in mitochondrial matrix and cross-references RHEA:31144.", "equation": "NAD+ + (S)-Hydroxyhexanoyl-CoA → NADH + H+ + 3-Oxohexanoyl-CoA", "inputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77322", "reactome_id": "R-ALL-77322", "label": "(S)-Hydroxyhexanoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62075", "formula": "C27H42N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77320", "reactome_id": "R-ALL-77320", "label": "3-Oxohexanoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62418", "formula": "C27H40N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of HADH dimer [mitochondrial matrix]", "activity_id": "71061", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:8687463", "title": "Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence.", "journal": "Biochem Biophys Res Commun", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8687463/" } ], "x": 1808.6666666666667, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77327", "label": "Hexanoyl-CoA+FAD =\u003e trans-Hex-2-enoyl-CoA+FADH2", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31208" ], "source_url": "https://reactome.org/content/detail/R-HSA-77327", "summary": "Hexanoyl-CoA+FAD =\u003e trans-Hex-2-enoyl-CoA+FADH2 is a Reactome release 97 curated reaction event. The source record connects FAD, HX-CoA, H+ to FADH2, trans-Hex-2-enoyl-CoA in mitochondrial matrix and cross-references RHEA:31208.", "equation": "FAD + HX-CoA + H+ → FADH2 + trans-Hex-2-enoyl-CoA", "inputs": [ { "node_id": "R-ALL-113596", "reactome_id": "R-ALL-113596", "label": "FAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57692", "formula": "C27H30N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77326", "reactome_id": "R-ALL-77326", "label": "HX-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62620", "formula": "C27H42N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31649", "reactome_id": "R-ALL-31649", "label": "FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58307", "formula": "C27H33N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77324", "reactome_id": "R-ALL-77324", "label": "trans-Hex-2-enoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62077", "formula": "C27H40N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acyl-CoA dehydrogenase activity of ACADS tetramer [mitochondrial matrix]", "activity_id": "77317", "ec_numbers": [ "1.3.99.3" ], "accessions": null } ], "citations": [ { "id": "PMID:13295225", "title": "On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. II. The electron-transferring flavoprotein.", "journal": "J Biol Chem", "year": 1956, "url": "https://pubmed.ncbi.nlm.nih.gov/13295225/" }, { "id": "PMID:3597357", "title": "Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver.", "journal": "J Biol Chem", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3597357/" } ], "x": 1251.3333333333333, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77331", "label": "(S)-Hydroxyoctanoyl-CoA+NAD =\u003e 3-Oxooctanoyl-CoA+NADH+H", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:31196" ], "source_url": "https://reactome.org/content/detail/R-HSA-77331", "summary": "(S)-Hydroxyoctanoyl-CoA+NAD =\u003e 3-Oxooctanoyl-CoA+NADH+H is a Reactome release 97 curated reaction event. The source record connects NAD+, (S)-Hydroxyoctanoyl-CoA to NADH, H+, 3-Oxooctanoyl-CoA in mitochondrial matrix and cross-references RHEA:31196.", "equation": "NAD+ + (S)-Hydroxyoctanoyl-CoA → NADH + H+ + 3-Oxooctanoyl-CoA", "inputs": [ { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77330", "reactome_id": "R-ALL-77330", "label": "(S)-Hydroxyoctanoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62617", "formula": "C29H46N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-77328", "reactome_id": "R-ALL-77328", "label": "3-Oxooctanoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62619", "formula": "C29H44N7O18P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of HADH dimer [mitochondrial matrix]", "activity_id": "71061", "ec_numbers": [ "1.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:8687463", "title": "Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence.", "journal": "Biochem Biophys Res Commun", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8687463/" } ], "x": 1530, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-77338", "label": "Octanoyl-CoA+FAD =\u003e trans-Oct-2-enoyl-CoA+FADH2", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "beta-oxidation", "fatty-acid-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:30944" ], "source_url": "https://reactome.org/content/detail/R-HSA-77338", "summary": "Octanoyl-CoA+FAD =\u003e trans-Oct-2-enoyl-CoA+FADH2 is a Reactome release 97 curated reaction event. The source record connects FAD, Octanoyl-CoA, H+ to FADH2, trans-Oct-2-enoyl-CoA in mitochondrial matrix and cross-references RHEA:30944.", "equation": "FAD + Octanoyl-CoA + H+ → FADH2 + trans-Oct-2-enoyl-CoA", "inputs": [ { "node_id": "R-ALL-113596", "reactome_id": "R-ALL-113596", "label": "FAD", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57692", "formula": "C27H30N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77337", "reactome_id": "R-ALL-77337", "label": "Octanoyl-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57386", "formula": "C29H46N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-31649", "reactome_id": "R-ALL-31649", "label": "FADH2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58307", "formula": "C27H33N9O15P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-77332", "reactome_id": "R-ALL-77332", "label": "trans-Oct-2-enoyl-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:62242", "formula": "C29H44N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acyl-CoA dehydrogenase activity of ACADM tetramer [mitochondrial matrix]", "activity_id": "77336", "ec_numbers": [ "1.3.99.3" ], "accessions": null } ], "citations": [ { "id": "PMID:13295225", "title": "On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. II. The electron-transferring flavoprotein.", "journal": "J Biol Chem", "year": 1956, "url": "https://pubmed.ncbi.nlm.nih.gov/13295225/" }, { "id": "PMID:3597357", "title": "Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver.", "journal": "J Biol Chem", "year": 1987, "url": "https://pubmed.ncbi.nlm.nih.gov/3597357/" } ], "x": 1808.6666666666667, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8847579", "label": "SCD5 desaturates ST-CoA to OLE-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "endoplasmic reticulum membrane · cytosol", "compartment_ids": [ "GO:0005789", "GO:0005829" ], "rhea_ids": [ "RHEA:19722" ], "source_url": "https://reactome.org/content/detail/R-HSA-8847579", "summary": "SCD5 desaturates ST-CoA to OLE-CoA is a Reactome release 97 curated reaction event. The source record connects ST-CoA, H+, O2 to OLE-CoA, Fe(3+)Cb5, H2O in endoplasmic reticulum membrane · cytosol and cross-references RHEA:19722.", "equation": "ST-CoA + 2 H+ + O2 + 2 Fe(2+)Cb5 → OLE-CoA + 2 Fe(3+)Cb5 + 2 H2O", "inputs": [ { "node_id": "R-ALL-428139", "reactome_id": "R-ALL-428139", "label": "ST-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57394", "formula": "C39H66N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-4085415", "reactome_id": "R-ALL-4085415", "label": "Fe(2+)Cb5", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:16518", "formula": "", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-5690558", "reactome_id": "R-ALL-5690558", "label": "OLE-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57387", "formula": "C39H64N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-4085425", "reactome_id": "R-ALL-4085425", "label": "Fe(3+)Cb5", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:18097", "formula": "", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "stearoyl-CoA 9-desaturase activity of SCD5 dimer [endoplasmic reticulum membrane]", "activity_id": "8847576", "ec_numbers": [ "1.14.19.1" ], "accessions": null } ], "citations": [ { "id": "PMID:15907797", "title": "Characterization of HSCD5, a novel human stearoyl-CoA desaturase unique to primates", "journal": "Biochem. Biophys. Res. Commun.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15907797/" }, { "id": "PMID:15610069", "title": "Characterization of human SCD2, an oligomeric desaturase with improved stability and enzyme activity by cross-linking in intact cells", "journal": "Biochem. J.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15610069/" } ], "x": 1808.6666666666667, "y": 1857.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8848338", "label": "PNPLA4 hydrolyzes TAG", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:12045" ], "source_url": "https://reactome.org/content/detail/R-HSA-8848338", "summary": "PNPLA4 hydrolyzes TAG is a Reactome release 97 curated reaction event. The source record connects TAG, H2O to DAG, LCFA(-), H+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:12045.", "equation": "TAG + H2O → DAG + LCFA(-) + H+", "inputs": [ { "node_id": "R-ALL-549167", "reactome_id": "R-ALL-549167", "label": "TAG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17855", "formula": "C6H5O6R3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76103", "reactome_id": "R-ALL-76103", "label": "DAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8876730", "reactome_id": "R-ALL-8876730", "label": "LCFA(-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57560", "formula": "CO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "triacylglycerol lipase activity of PNPLA4 [cytosol]", "activity_id": "8848353", "ec_numbers": [ "3.1.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:17603008", "title": "A comparative study of human GS2, its paralogues, and its rat orthologue", "journal": "Biochem. Biophys. Res. Commun.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17603008/" }, { "id": "PMID:15364929", "title": "Identification, cloning, expression, and purification of three novel human calcium-independent phospholipase A2 family members possessing triacylglycerol lipase and acylglycerol transacylase activities", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15364929/" } ], "x": 3600, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8848339", "label": "PNPLA5 hydrolyzes TAG", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:12045" ], "source_url": "https://reactome.org/content/detail/R-HSA-8848339", "summary": "PNPLA5 hydrolyzes TAG is a Reactome release 97 curated reaction event. The source record connects TAG, H2O to DAG, LCFA(-), H+ in cytosol · endoplasmic reticulum membrane and cross-references RHEA:12045.", "equation": "TAG + H2O → DAG + LCFA(-) + H+", "inputs": [ { "node_id": "R-ALL-549167", "reactome_id": "R-ALL-549167", "label": "TAG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17855", "formula": "C6H5O6R3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-76103", "reactome_id": "R-ALL-76103", "label": "DAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17815", "formula": "C5H6O5R2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8876730", "reactome_id": "R-ALL-8876730", "label": "LCFA(-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57560", "formula": "CO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "triacylglycerol lipase activity of PNPLA5 [cytosol]", "activity_id": "8848358", "ec_numbers": [ "3.1.1.3" ], "accessions": null } ], "citations": [ { "id": "PMID:17603008", "title": "A comparative study of human GS2, its paralogues, and its rat orthologue", "journal": "Biochem. Biophys. Res. Commun.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17603008/" } ], "x": 3878.666666666667, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8848355", "label": "PNPLA4 hydrolyzes retinyl palmitate", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "triglycerides" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13934" ], "source_url": "https://reactome.org/content/detail/R-HSA-8848355", "summary": "PNPLA4 hydrolyzes retinyl palmitate is a Reactome release 97 curated reaction event. The source record connects atR-PALM, H2O to atROL, PALM(-), H+ in cytosol and cross-references RHEA:13934.", "equation": "atR-PALM + H2O → atROL + PALM(-) + H+", "inputs": [ { "node_id": "R-ALL-2534407", "reactome_id": "R-ALL-2534407", "label": "atR-PALM", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17616", "formula": "C36H60O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-30209", "reactome_id": "R-ALL-30209", "label": "atROL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17336", "formula": "C20H30O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8848337", "reactome_id": "R-ALL-8848337", "label": "PALM(-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:7896", "formula": "C16H31O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "retinyl-palmitate esterase activity of PNPLA4 [cytosol]", "activity_id": "8848341", "ec_numbers": [ "3.1.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:16741517", "title": "Molecular screening for GS2 lipase regulators: inhibition of keratinocyte retinylester hydrolysis by TIP47", "journal": "J. Invest. Dermatol.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16741517/" }, { "id": "PMID:17603008", "title": "A comparative study of human GS2, its paralogues, and its rat orthologue", "journal": "Biochem. Biophys. Res. Commun.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17603008/" }, { "id": "PMID:19181555", "title": "GS2 as a retinol transacylase and as a catalytic dyad independent regulator of retinylester accretion", "journal": "Mol. Genet. Metab.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19181555/" } ], "x": 3321.3333333333335, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8851089", "label": "NTPDase2 hydrolyzes nucleoside triphosphates", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "extracellular region · plasma membrane", "compartment_ids": [ "GO:0005576", "GO:0005886" ], "rhea_ids": [ "RHEA:13066", "RHEA:19670", "RHEA:29388", "RHEA:28331", "RHEA:64901" ], "source_url": "https://reactome.org/content/detail/R-HSA-8851089", "summary": "NTPDase2 hydrolyzes nucleoside triphosphates is a Reactome release 97 curated reaction event. The source record connects ATP,GTP,CTP,ITP,UTP, H2O to ADP,GDP,CDP,IDP,UDP, Pi, H+ in extracellular region · plasma membrane and cross-references RHEA:13066, RHEA:19670, RHEA:29388, RHEA:28331, RHEA:64901.", "equation": "ATP,GTP,CTP,ITP,UTP + H2O → ADP,GDP,CDP,IDP,UDP + Pi + H+", "inputs": [ { "node_id": "R-ALL-8936166", "reactome_id": "R-ALL-8936166", "label": "ATP,GTP,CTP,ITP,UTP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-8851092", "reactome_id": "R-ALL-8851092", "label": "ADP,GDP,CDP,IDP,UDP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-109277", "reactome_id": "R-ALL-109277", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ribonucleoside triphosphate phosphatase activity of NTPDase2:Ca2+,Mg2+ [plasma membrane]", "activity_id": "8851096", "ec_numbers": [ "3.6.1.15" ], "accessions": null } ], "citations": [ { "id": "PMID:10510450", "title": "Functional expression of a cDNA encoding a human ecto-ATPase", "journal": "Br. J. Pharmacol.", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10510450/" }, { "id": "PMID:12888562", "title": "Requirement of Cys399 for processing of the human ecto-ATPase (NTPDase2) and its implications for determination of the activities of splice variants of the enzyme", "journal": "J. Biol. Chem.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12888562/" }, { "id": "PMID:12694193", "title": "Determination of native oligomeric state and substrate specificity of rat NTPDase1 and NTPDase2 after heterologous expression in Xenopus oocytes", "journal": "Eur. J. Biochem.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12694193/" }, { "id": "PMID:8995405", "title": "Complementary DNA cloning and sequencing of the chicken muscle ecto-ATPase. Homology with the lymphoid cell activation antigen CD39", "journal": "J. Biol. Chem.", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/8995405/" }, { "id": "PMID:9364474", "title": "An ecto-ATPase and an ecto-ATP diphosphohydrolase are expressed in rat brain", "journal": "Neuropharmacology", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9364474/" }, { "id": "PMID:11929769", "title": "Differential catalytic properties and vascular topography of murine nucleoside triphosphate diphosphohydrolase 1 (NTPDase1) and NTPDase2 have implications for thromboregulation", "journal": "Blood", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11929769/" } ], "x": 2381.3333333333335, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8851494", "label": "NTPDase7 hydrolyzes nucleoside triphosphates", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "endocytic vesicle lumen · endocytic vesicle membrane", "compartment_ids": [ "GO:0071682", "GO:0030666" ], "rhea_ids": [ "RHEA:29388", "RHEA:64901", "RHEA:19670" ], "source_url": "https://reactome.org/content/detail/R-HSA-8851494", "summary": "NTPDase7 hydrolyzes nucleoside triphosphates is a Reactome release 97 curated reaction event. The source record connects CTP,GTP,UTP, H2O to CDP,GDP,UDP, Pi, H+ in endocytic vesicle lumen · endocytic vesicle membrane and cross-references RHEA:29388, RHEA:64901, RHEA:19670.", "equation": "CTP,GTP,UTP + H2O → CDP,GDP,UDP + Pi + H+", "inputs": [ { "node_id": "R-ALL-8851509", "reactome_id": "R-ALL-8851509", "label": "CTP,GTP,UTP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "endocytic vesicle lumen", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-8851517", "reactome_id": "R-ALL-8851517", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "endocytic vesicle lumen", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-8851518", "reactome_id": "R-ALL-8851518", "label": "CDP,GDP,UDP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "endocytic vesicle lumen", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-8851513", "reactome_id": "R-ALL-8851513", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "endocytic vesicle lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-5228597", "reactome_id": "R-ALL-5228597", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "endocytic vesicle lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ribonucleoside triphosphate phosphatase activity of NTPDase7:Ca2+ [endocytic vesicle membrane]", "activity_id": "8851507", "ec_numbers": [ "3.6.1.15" ], "accessions": null } ], "citations": [ { "id": "PMID:11278936", "title": "Molecular cloning and characterization of a novel mammalian endo-apyrase (LALP1)", "journal": "J. Biol. Chem.", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11278936/" }, { "id": "PMID:23241884", "title": "Ecto-nucleoside triphosphate diphosphohydrolase 7 controls Th17 cell responses through regulation of luminal ATP in the small intestine", "journal": "J. Immunol.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23241884/" } ], "x": 2660, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8851538", "label": "NTPDase8 hydrolyzes nucleoside triphosphates", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "extracellular region · plasma membrane", "compartment_ids": [ "GO:0005576", "GO:0005886" ], "rhea_ids": [ "RHEA:13066", "RHEA:19670", "RHEA:28331", "RHEA:29388", "RHEA:19014", "RHEA:64901" ], "source_url": "https://reactome.org/content/detail/R-HSA-8851538", "summary": "NTPDase8 hydrolyzes nucleoside triphosphates is a Reactome release 97 curated reaction event. The source record connects H2O, ATP,GTP,ITP,CTP,TTP,UTP to Pi, ADP,GDP,IDP,CDP,TDP,UDP, H+ in extracellular region · plasma membrane and cross-references RHEA:13066, RHEA:19670, RHEA:28331, RHEA:29388, RHEA:19014, RHEA:64901.", "equation": "H2O + ATP,GTP,ITP,CTP,TTP,UTP → Pi + ADP,GDP,IDP,CDP,TDP,UDP + H+", "inputs": [ { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9726184", "reactome_id": "R-ALL-9726184", "label": "ATP,GTP,ITP,CTP,TTP,UTP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-109277", "reactome_id": "R-ALL-109277", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9726189", "reactome_id": "R-ALL-9726189", "label": "ADP,GDP,IDP,CDP,TDP,UDP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ribonucleoside triphosphate phosphatase activity of NTPDase8:Ca2+,Mg2+ [plasma membrane]", "activity_id": "8851543", "ec_numbers": [ "3.6.1.15" ], "accessions": null } ], "citations": [ { "id": "PMID:17095758", "title": "Cloning, purification, and identification of the liver canalicular ecto-ATPase as NTPDase8", "journal": "Am. J. Physiol. Gastrointest. Liver Physiol.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17095758/" }, { "id": "PMID:10681547", "title": "Identification and characterization of a novel hepatic canalicular ATP diphosphohydrolase", "journal": "J. Biol. Chem.", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10681547/" } ], "x": 2938.666666666667, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8851550", "label": "NTPDase8 hydrolyzes nucleoside diphosphates", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-catabolism" ], "compartment": "extracellular region · plasma membrane", "compartment_ids": [ "GO:0005576", "GO:0005886" ], "rhea_ids": [ "RHEA:64961", "RHEA:61437", "RHEA:22157", "RHEA:35208", "RHEA:64881", "RHEA:64877" ], "source_url": "https://reactome.org/content/detail/R-HSA-8851550", "summary": "NTPDase8 hydrolyzes nucleoside diphosphates is a Reactome release 97 curated reaction event. The source record connects H2O, ADP,GDP,IDP,CDP,TDP,UDP to Pi, AMP,GMP,IMP,CMP,TMP,UMP, H+ in extracellular region · plasma membrane and cross-references RHEA:64961, RHEA:61437, RHEA:22157, RHEA:35208, RHEA:64881, RHEA:64877.", "equation": "H2O + ADP,GDP,IDP,CDP,TDP,UDP → Pi + AMP,GMP,IMP,CMP,TMP,UMP + H+", "inputs": [ { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9726189", "reactome_id": "R-ALL-9726189", "label": "ADP,GDP,IDP,CDP,TDP,UDP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-109277", "reactome_id": "R-ALL-109277", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9726186", "reactome_id": "R-ALL-9726186", "label": "AMP,GMP,IMP,CMP,TMP,UMP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside diphosphate phosphatase activity of NTPDase8:Ca2+,Mg2+ [plasma membrane]", "activity_id": "8851544", "ec_numbers": [ "3.6.1.6" ], "accessions": null } ], "citations": [ { "id": "PMID:17095758", "title": "Cloning, purification, and identification of the liver canalicular ecto-ATPase as NTPDase8", "journal": "Am. J. Physiol. Gastrointest. Liver Physiol.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17095758/" }, { "id": "PMID:10681547", "title": "Identification and characterization of a novel hepatic canalicular ATP diphosphohydrolase", "journal": "J. Biol. Chem.", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10681547/" }, { "id": "PMID:15122917", "title": "Cloning and characterization of mouse nucleoside triphosphate diphosphohydrolase-8", "journal": "Biochemistry", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15122917/" } ], "x": 2381.3333333333335, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8853391", "label": "GlcNAc (Man)5 translocates from cytosol to lysosomal lumen", "schema_class": "BlackBoxEvent", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "oligosaccharide-recovery" ], "compartment": "cytosol · lysosomal lumen", "compartment_ids": [ "GO:0005829", "GO:0043202" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-8853391", "summary": "GlcNAc (Man)5 translocates from cytosol to lysosomal lumen is a Reactome release 97 curated black-box event whose internal chemistry is not expanded by the source record. The source record connects alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc to alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc in cytosol · lysosomal lumen.", "equation": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc → alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc", "inputs": [ { "node_id": "R-ALL-6799543", "reactome_id": "R-ALL-6799543", "label": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:91280", "formula": "C38H65NO31", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-8853396", "reactome_id": "R-ALL-8853396", "label": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:91280", "formula": "C38H65NO31", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [], "citations": [ { "id": "PMID:9008702", "title": "Transfer of free polymannose-type oligosaccharides from the cytosol to lysosomes in cultured human hepatocellular carcinoma HepG2 cells", "journal": "J. Cell Biol.", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9008702/" } ], "x": 3600, "y": 1033.75, "lod": "pathway", "collapsed": true, "transport": true }, { "id": "R-HSA-8853686", "label": "MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "oligosaccharide-recovery" ], "compartment": "lysosomal lumen", "compartment_ids": [ "GO:0043202" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-8853686", "summary": "MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 is a Reactome release 97 curated reaction event. The source record connects alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc, H2O to alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc, Man in lysosomal lumen.", "equation": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc + H2O → alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc + 2 Man", "inputs": [ { "node_id": "R-ALL-8853396", "reactome_id": "R-ALL-8853396", "label": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:91280", "formula": "C38H65NO31", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1605715", "reactome_id": "R-ALL-1605715", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-6799577", "reactome_id": "R-ALL-6799577", "label": "alpha-D-Man-(1-\u003e3)-[alpha-D-Man-(1-\u003e6)]-beta-D-Man-(1-\u003e4)-beta-D-GlcNAc", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:91279", "formula": "C26H45NO21", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-6799594", "reactome_id": "R-ALL-6799594", "label": "Man", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:4208", "formula": "C6H12O6", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "alpha-mannosidase activity of MAN2B1:Zn2+ [lysosomal lumen]", "activity_id": "8853698", "ec_numbers": [ "3.2.1.24" ], "accessions": null } ], "citations": [ { "id": "PMID:8910458", "title": "Cloning, expression, purification, and characterization of the human broad specificity lysosomal acid alpha-mannosidase", "journal": "J. Biol. Chem.", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8910458/" }, { "id": "PMID:11350179", "title": "Purification and characterization of recombinant human lysosomal alpha-mannosidase", "journal": "Mol. Genet. Metab.", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11350179/" }, { "id": "PMID:10513892", "title": "Lysosomal alpha-D-mannosidase", "journal": "Biosci. Rep.", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10513892/" }, { "id": "PMID:25762455", "title": "amamutdb.no: A relational database for MAN2B1 allelic variants that compiles genotypes, clinical phenotypes, and biochemical and structural data of mutant MAN2B1 in α-mannosidosis", "journal": "Hum. Mutat.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25762455/" }, { "id": "PMID:25345101", "title": "Alpha-mannosidosis - a review of genetic, clinical findings and options of treatment", "journal": "Pediatr Endocrinol Rev", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/25345101/" } ], "x": 3878.666666666667, "y": 839.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8853710", "label": "MANBA hydrolyses GlcNAc:Man", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "oligosaccharide-recovery" ], "compartment": "lysosomal lumen", "compartment_ids": [ "GO:0043202" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-8853710", "summary": "MANBA hydrolyses GlcNAc:Man is a Reactome release 97 curated reaction event. The source record connects GlcNAc:Man to Man, GlcNAc in lysosomal lumen.", "equation": "GlcNAc:Man → Man + GlcNAc", "inputs": [ { "node_id": "R-ALL-8853719", "reactome_id": "R-ALL-8853719", "label": "GlcNAc:Man", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:71553", "formula": "C14H25NO11", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-6799594", "reactome_id": "R-ALL-6799594", "label": "Man", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:4208", "formula": "C6H12O6", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1678743", "reactome_id": "R-ALL-1678743", "label": "GlcNAc", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17411", "formula": "C8H15NO6", "compartment": "lysosomal lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "beta-mannosidase activity of MANBA [lysosomal lumen]", "activity_id": "8853695", "ec_numbers": [ "3.2.1.25" ], "accessions": null } ], "citations": [ { "id": "PMID:9384606", "title": "Human beta-mannosidase cDNA characterization and first identification of a mutation associated with human beta-mannosidosis", "journal": "Hum. Mol. Genet.", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9384606/" }, { "id": "PMID:1576208", "title": "Mammalian beta-D-mannosidase and beta-mannosidosis", "journal": "Biochimie", "year": 1992, "url": "https://pubmed.ncbi.nlm.nih.gov/1576208/" }, { "id": "PMID:17420068", "title": "Angiokeratoma corporis diffusum in human beta-mannosidosis: Report of a new case and a novel mutation", "journal": "J. Am. Acad. Dermatol.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17420068/" } ], "x": 3321.3333333333335, "y": 1033.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8863761", "label": "UXS1 tetramer decarboxylates UDP-glucuronate to UDP-xylose", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "biological-oxidations", "glycosaminoglycans", "phase-two" ], "compartment": "Golgi lumen · Golgi membrane", "compartment_ids": [ "GO:0005796", "GO:0000139" ], "rhea_ids": [ "RHEA:23917" ], "source_url": "https://reactome.org/content/detail/R-HSA-8863761", "summary": "UXS1 tetramer decarboxylates UDP-glucuronate to UDP-xylose is a Reactome release 97 curated reaction event. The source record connects UDP-GlcA, H+ to UDP-Xyl, CO2 in Golgi lumen · Golgi membrane and cross-references RHEA:23917.", "equation": "UDP-GlcA + H+ → UDP-Xyl + CO2", "inputs": [ { "node_id": "R-ALL-1889970", "reactome_id": "R-ALL-1889970", "label": "UDP-GlcA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58052", "formula": "C15H19N2O18P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-425969", "reactome_id": "R-ALL-425969", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-742361", "reactome_id": "R-ALL-742361", "label": "UDP-Xyl", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57632", "formula": "C14H20N2O16P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8863760", "reactome_id": "R-ALL-8863760", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "UDP-glucuronate decarboxylase activity of UXS1 tetramer [Golgi membrane]", "activity_id": "8863767", "ec_numbers": [ "4.1.1.35" ], "accessions": null } ], "citations": [ { "id": "PMID:22810237", "title": "Structure and mechanism of human UDP-xylose synthase: evidence for a promoting role of sugar ring distortion in a three-step catalytic conversion of UDP-glucuronic acid", "journal": "J Biol Chem", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22810237/" }, { "id": "PMID:11877387", "title": "UDP-glucuronate decarboxylase, a key enzyme in proteoglycan synthesis: cloning, characterization, and localization", "journal": "J. Biol. Chem.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11877387/" }, { "id": "PMID:23656592", "title": "Human UDP-α-d-xylose synthase forms a catalytically important tetramer that has not been observed in crystal structures", "journal": "Biochemistry", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23656592/" }, { "id": "PMID:25521717", "title": "Man o' war mutation in UDP-α-D-xylose synthase favors the abortive catalytic cycle and uncovers a latent potential for hexamer formation", "journal": "Biochemistry", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25521717/" } ], "x": 2938.666666666667, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8866941", "label": "MID1IP1 binds THRSP", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-8866941", "summary": "MID1IP1 binds THRSP is a Reactome release 97 curated reaction event. The source record connects MID1IP1, THRSP to MID1IP1:THRSP in cytosol.", "equation": "MID1IP1 + THRSP → MID1IP1:THRSP", "inputs": [ { "node_id": "R-HSA-8867328", "reactome_id": "R-HSA-8867328", "label": "MID1IP1", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "EntityWithAccessionedSequence", "currency": false }, { "node_id": "R-HSA-8867326", "reactome_id": "R-HSA-8867326", "label": "THRSP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "outputs": [ { "node_id": "R-HSA-8867329", "reactome_id": "R-HSA-8867329", "label": "MID1IP1:THRSP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "Complex", "currency": false } ], "catalysts": [ { "label": "molecular function activator activity of THRSP [cytosol]", "activity_id": "9954170", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:18556348", "title": "Hepatic expression of the SPOT 14 (S14) paralog S14-related (Mid1 interacting protein) is regulated by dietary carbohydrate", "journal": "Endocrinology", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18556348/" }, { "id": "PMID:20233797", "title": "Overlapping roles of the glucose-responsive genes, S14 and S14R, in hepatic lipogenesis", "journal": "Endocrinology", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20233797/" }, { "id": "PMID:24277613", "title": "Spot14/Mig12 heterocomplex sequesters polymerization and restrains catalytic function of human acetyl-CoA carboxylase 2", "journal": "J. Mol. Recognit.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/24277613/" } ], "x": 590, "y": 2336.1666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8869606", "label": "NMRK1 phosphorylates NAR to yield NAMN", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:25569" ], "source_url": "https://reactome.org/content/detail/R-HSA-8869606", "summary": "NMRK1 phosphorylates NAR to yield NAMN is a Reactome release 97 curated reaction event. The source record connects NAR, ATP to ADP, H+, NAMN in cytosol and cross-references RHEA:25569.", "equation": "NAR + ATP → ADP + H+ + NAMN", "inputs": [ { "node_id": "R-ALL-8869365", "reactome_id": "R-ALL-8869365", "label": "NAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58527", "formula": "C11H13NO6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-8869359", "reactome_id": "R-ALL-8869359", "label": "NAMN", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57502", "formula": "C11H12NO9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "nicotinate riboside kinase activity of NMRK1 [cytosol]", "activity_id": "8869636", "ec_numbers": [ "2.7.1.173" ], "accessions": null } ], "citations": [ { "id": "PMID:21504897", "title": "Pathways and subcellular compartmentation of NAD biosynthesis in human cells: from entry of extracellular precursors to mitochondrial NAD generation", "journal": "J. Biol. Chem.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21504897/" }, { "id": "PMID:19531469", "title": "The intrinsic reactivity of ATP and the catalytic proficiencies of kinases acting on glucose, N-acetylgalactosamine, and homoserine: a thermodynamic analysis", "journal": "J. Biol. Chem.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19531469/" }, { "id": "PMID:17914902", "title": "Nicotinamide riboside kinase structures reveal new pathways to NAD+", "journal": "PLoS Biol.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17914902/" } ], "x": 5391.333333333333, "y": 3143.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8875071", "label": "ACSS3 ligates CoA to CH3COO-", "schema_class": "Reaction", "domain_id": "metabolic-integration", "pathway_ids": [ "ketone-bodies" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:23177" ], "source_url": "https://reactome.org/content/detail/R-HSA-8875071", "summary": "ACSS3 ligates CoA to CH3COO- is a Reactome release 97 curated reaction event. The source record connects CH3COO-, ATP, CoA-SH to Ac-CoA, AMP, PPi in mitochondrial matrix and cross-references RHEA:23177.", "equation": "CH3COO- + ATP + CoA-SH → Ac-CoA + AMP + PPi", "inputs": [ { "node_id": "R-ALL-113539", "reactome_id": "R-ALL-113539", "label": "CH3COO-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30089", "formula": "C2H3O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113559", "reactome_id": "R-ALL-113559", "label": "Ac-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159448", "reactome_id": "R-ALL-159448", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "acetyl-CoA synthetase activity of ACSS3 [mitochondrial matrix]", "activity_id": "8875070", "ec_numbers": [ "6.2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:17762044", "title": "Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome", "journal": "J. Lipid Res.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17762044/" } ], "x": 7740, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8875077", "label": "SLC27A3 ligates CoA-SH to VLCFA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:54537" ], "source_url": "https://reactome.org/content/detail/R-HSA-8875077", "summary": "SLC27A3 ligates CoA-SH to VLCFA is a Reactome release 97 curated reaction event. The source record connects ATP, CoA-SH, VLCFA to VLCFA-CoA, AMP, PPi in mitochondrial matrix and cross-references RHEA:54537.", "equation": "ATP + CoA-SH + VLCFA → VLCFA-CoA + AMP + PPi", "inputs": [ { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29374", "reactome_id": "R-ALL-29374", "label": "CoA-SH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-5696008", "reactome_id": "R-ALL-5696008", "label": "VLCFA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58950", "formula": "CO2R", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-5696003", "reactome_id": "R-ALL-5696003", "label": "VLCFA-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:138261", "formula": "C22H31N7O17P3SR", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-159448", "reactome_id": "R-ALL-159448", "label": "AMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456215", "formula": "C10H12N5O7P", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-159450", "reactome_id": "R-ALL-159450", "label": "PPi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33019", "formula": "HO7P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "very long-chain fatty acid-CoA ligase activity of SLC27A3 [mitochondrial matrix]", "activity_id": "8875075", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:17762044", "title": "Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome", "journal": "J. Lipid Res.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17762044/" }, { "id": "PMID:15469937", "title": "Mouse very long-chain Acyl-CoA synthetase 3/fatty acid transport protein 3 catalyzes fatty acid activation but not fatty acid transport in MA-10 cells", "journal": "J. Biol. Chem.", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15469937/" } ], "x": 1808.6666666666667, "y": 1597.8333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8875623", "label": "SLC25A18,22 import L-Glu, H+", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport", "malate-aspartate-shuttle" ], "compartment": "cytosol · mitochondrial inner membrane · mitochondrial matrix", "compartment_ids": [ "GO:0005829", "GO:0005743", "GO:0005759" ], "rhea_ids": [ "RHEA:70955", "RHEA:70957" ], "source_url": "https://reactome.org/content/detail/R-HSA-8875623", "summary": "SLC25A18,22 import L-Glu, H+ is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects L-Glu, H+ to L-Glu, H+ in cytosol · mitochondrial inner membrane · mitochondrial matrix and cross-references RHEA:70955, RHEA:70957.", "equation": "L-Glu + H+ → L-Glu + H+", "inputs": [ { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "amino acid:proton symporter activity of SLC25A18,A22 [mitochondrial inner membrane]", "activity_id": "8875618", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:11897791", "title": "Identification of the mitochondrial glutamate transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms", "journal": "J. Biol. Chem.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/11897791/" }, { "id": "PMID:15592994", "title": "Impaired mitochondrial glutamate transport in autosomal recessive neonatal myoclonic epilepsy", "journal": "Am. J. Hum. Genet.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15592994/" }, { "id": "PMID:34679360", "title": "Neonatal Seizures: An Overview of Genetic Causes and Treatment Options", "journal": "Brain Sci", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/34679360/" } ], "x": 4730, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-8876889", "label": "Btn-ACACB:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoA", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "cytosol · mitochondrial outer membrane", "compartment_ids": [ "GO:0005829", "GO:0005741" ], "rhea_ids": [ "RHEA:11309" ], "source_url": "https://reactome.org/content/detail/R-HSA-8876889", "summary": "Btn-ACACB:2Mn2+ polymer carboxylates Ac-CoA to form Mal-CoA is a Reactome release 97 curated reaction event. The source record connects Ac-CoA, ATP, HCO3- to ADP, Pi, Mal-CoA in cytosol · mitochondrial outer membrane and cross-references RHEA:11309.", "equation": "Ac-CoA + ATP + HCO3- → ADP + Pi + Mal-CoA + H+", "inputs": [ { "node_id": "R-ALL-76183", "reactome_id": "R-ALL-76183", "label": "Ac-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57288", "formula": "C23H34N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-111627", "reactome_id": "R-ALL-111627", "label": "HCO3-", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17544", "formula": "CHO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29508", "reactome_id": "R-ALL-29508", "label": "Mal-CoA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57384", "formula": "C24H33N7O19P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "acetyl-CoA carboxylase activity of Btn-ACACB:2Mn2+ polymer [mitochondrial outer membrane]", "activity_id": "8876888", "ec_numbers": [ "6.4.1.2" ], "accessions": null } ], "citations": [ { "id": "PMID:17223360", "title": "Expression, purification, and characterization of human acetyl-CoA carboxylase 2", "journal": "Protein Expr Purif", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17223360/" }, { "id": "PMID:10677481", "title": "The subcellular localization of acetyl-CoA carboxylase 2", "journal": "Proc Natl Acad Sci U S A", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10677481/" }, { "id": "PMID:20457939", "title": "Induced polymerization of mammalian acetyl-CoA carboxylase by MIG12 provides a tertiary level of regulation of fatty acid synthesis", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20457939/" }, { "id": "PMID:22123817", "title": "Selectivity in post-translational biotin addition to five human carboxylases", "journal": "J. Biol. Chem.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22123817/" }, { "id": "PMID:18556348", "title": "Hepatic expression of the SPOT 14 (S14) paralog S14-related (Mid1 interacting protein) is regulated by dietary carbohydrate", "journal": "Endocrinology", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18556348/" }, { "id": "PMID:24277613", "title": "Spot14/Mig12 heterocomplex sequesters polymerization and restrains catalytic function of human acetyl-CoA carboxylase 2", "journal": "J. Mol. Recognit.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/24277613/" } ], "x": 311.33333333333337, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8878787", "label": "ALPI dimer hydrolyses phosphate monoesters", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "extracellular region · plasma membrane", "compartment_ids": [ "GO:0005576", "GO:0005886" ], "rhea_ids": [ "RHEA:15018" ], "source_url": "https://reactome.org/content/detail/R-HSA-8878787", "summary": "ALPI dimer hydrolyses phosphate monoesters is a Reactome release 97 curated reaction event. The source record connects H2O, phosphate monoester to Pi, Alcohol in extracellular region · plasma membrane and cross-references RHEA:15018.", "equation": "H2O + phosphate monoester → Pi + Alcohol", "inputs": [ { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-8878780", "reactome_id": "R-ALL-8878780", "label": "phosphate monoester", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:7794", "formula": "H2O4PR", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-109277", "reactome_id": "R-ALL-109277", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-8878776", "reactome_id": "R-ALL-8878776", "label": "Alcohol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:30879", "formula": "HOR", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "alkaline phosphatase activity of ALPI:2Ca2+:Mg2+ dimer [plasma membrane]", "activity_id": "8878778", "ec_numbers": [ "3.1.3.1" ], "accessions": null } ], "citations": [ { "id": "PMID:3458202", "title": "Partial sequencing of human adult, human fetal, and bovine intestinal alkaline phosphatases: comparison with the human placental and liver isozymes", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 1986, "url": "https://pubmed.ncbi.nlm.nih.gov/3458202/" }, { "id": "PMID:24966474", "title": "Alkaline phosphatase: an overview", "journal": "Indian J Clin Biochem", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24966474/" } ], "x": 2381.3333333333335, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8878815", "label": "Formation of 4Fe-4S cluster on ISCA1:ISCA2", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "iron-sulfur" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [], "source_url": "https://reactome.org/content/detail/R-HSA-8878815", "summary": "Formation of 4Fe-4S cluster on ISCA1:ISCA2 is a Reactome release 97 curated reaction event. The source record connects GLRX5:2Fe-2S, ISCA1:ISCA2 to ISCA1:ISCA2:4Fe-4S, GLRX5 in mitochondrial matrix.", "equation": "2 GLRX5:2Fe-2S + ISCA1:ISCA2 → ISCA1:ISCA2:4Fe-4S + 2 GLRX5", "inputs": [ { "node_id": "R-HSA-8878793", "reactome_id": "R-HSA-8878793", "label": "GLRX5:2Fe-2S", "role": "input", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-HSA-8878840", "reactome_id": "R-HSA-8878840", "label": "ISCA1:ISCA2", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false } ], "outputs": [ { "node_id": "R-HSA-8878796", "reactome_id": "R-HSA-8878796", "label": "ISCA1:ISCA2:4Fe-4S", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "Complex", "currency": false }, { "node_id": "R-HSA-2564761", "reactome_id": "R-HSA-2564761", "label": "GLRX5", "role": "output", "stoichiometry": 2, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "EntityWithAccessionedSequence", "currency": false } ], "catalysts": [ { "label": "iron-sulfur transferase activity of ISCA1:ISCA2 [mitochondrial matrix]", "activity_id": "9954150", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:25347204", "title": "Formation of [4Fe-4S] clusters in the mitochondrial iron-sulfur cluster assembly machinery", "journal": "J. Am. Chem. Soc.", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/25347204/" }, { "id": "PMID:22323289", "title": "The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation", "journal": "Mol. Biol. Cell", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22323289/" }, { "id": "PMID:24733926", "title": "[2Fe-2S] cluster transfer in iron-sulfur protein biogenesis", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24733926/" }, { "id": "PMID:20668094", "title": "Characterization of the human HSC20, an unusual DnaJ type III protein, involved in iron-sulfur cluster biogenesis", "journal": "Hum. Mol. Genet.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20668094/" }, { "id": "PMID:21987576", "title": "Specialized function of yeast Isa1 and Isa2 proteins in the maturation of mitochondrial [4Fe-4S] proteins", "journal": "J. Biol. Chem.", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21987576/" }, { "id": "PMID:27989128", "title": "[4Fe-4S] Cluster Assembly in Mitochondria and Its Impairment by Copper", "journal": "J. Am. Chem. Soc.", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/27989128/" } ], "x": 5670, "y": 3573.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8932980", "label": "SERINC3,5,(1,2,4) transport L-Ser from cytosol to plasma membrane", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol · plasma membrane", "compartment_ids": [ "GO:0005829", "GO:0005886" ], "rhea_ids": [ "RHEA:35031" ], "source_url": "https://reactome.org/content/detail/R-HSA-8932980", "summary": "SERINC3,5,(1,2,4) transport L-Ser from cytosol to plasma membrane is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects L-Ser to L-Ser in cytosol · plasma membrane and cross-references RHEA:35031.", "equation": "L-Ser → L-Ser", "inputs": [ { "node_id": "R-ALL-200736", "reactome_id": "R-ALL-200736", "label": "L-Ser", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:33384", "formula": "C3H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-8932979", "reactome_id": "R-ALL-8932979", "label": "L-Ser", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33384", "formula": "C3H7NO3", "compartment": "plasma membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "molecular carrier activity of SERINC3,5,(1,2,4) [plasma membrane]", "activity_id": "8932974", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:26703715", "title": "Spotlight on HIV-1 Nef: SERINC3 and SERINC5 Identified as Restriction Factors Antagonized by the Pathogenesis Factor", "journal": "Viruses", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/26703715/" }, { "id": "PMID:16120614", "title": "Serinc, an activity-regulated protein family, incorporates serine into membrane lipid synthesis", "journal": "J Biol Chem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16120614/" }, { "id": "PMID:26416733", "title": "SERINC3 and SERINC5 restrict HIV-1 infectivity and are counteracted by Nef", "journal": "Nature", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/26416733/" } ], "x": 590, "y": 2994.1111111111113, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-893583", "label": "kynurenine + 2-oxoglutarate =\u003e 4-(2-aminophenyl)-2,4-dioxobutanoic acid + glutamate", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:20965" ], "source_url": "https://reactome.org/content/detail/R-HSA-893583", "summary": "kynurenine + 2-oxoglutarate =\u003e 4-(2-aminophenyl)-2,4-dioxobutanoic acid + glutamate is a Reactome release 97 curated reaction event. The source record connects L-KYN, 2OG to APDBA, L-Glu in mitochondrial matrix and cross-references RHEA:20965.", "equation": "L-KYN + 2OG → APDBA + L-Glu", "inputs": [ { "node_id": "R-ALL-893607", "reactome_id": "R-ALL-893607", "label": "L-KYN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57959", "formula": "C10H12N2O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113594", "reactome_id": "R-ALL-113594", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-893617", "reactome_id": "R-ALL-893617", "label": "APDBA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58147", "formula": "C10H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113552", "reactome_id": "R-ALL-113552", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-kynurenine:2-oxoglutarate transaminase activity of AADAT dimer [mitochondrial matrix]", "activity_id": "893618", "ec_numbers": [ "2.6.1.7" ], "accessions": null } ], "citations": [ { "id": "PMID:18056995", "title": "Crystal structure of human kynurenine aminotransferase II", "journal": "J Biol Chem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18056995/" }, { "id": "PMID:13069505", "title": "The transamination of kynurenine", "journal": "J Biol Chem", "year": 1953, "url": "https://pubmed.ncbi.nlm.nih.gov/13069505/" } ], "x": 7740, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-893593", "label": "phenylalanine + pyruvate =\u003e keto-phenylpyruvate + alanine", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "phenylalanine-tyrosine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13054" ], "source_url": "https://reactome.org/content/detail/R-HSA-893593", "summary": "phenylalanine + pyruvate =\u003e keto-phenylpyruvate + alanine is a Reactome release 97 curated reaction event. The source record connects L-Phe, PYR to L-Ala, kPPV in cytosol and cross-references RHEA:13054.", "equation": "L-Phe + PYR → L-Ala + kPPV", "inputs": [ { "node_id": "R-ALL-29502", "reactome_id": "R-ALL-29502", "label": "L-Phe", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58095", "formula": "C9H11NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29432", "reactome_id": "R-ALL-29432", "label": "L-Ala", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57972", "formula": "C3H7NO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-2160487", "reactome_id": "R-ALL-2160487", "label": "kPPV", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18005", "formula": "C9H7O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-phenylalanine:pyruvate transaminase activity of PXLP-KYAT1 dimer [cytosol]", "activity_id": "893585", "ec_numbers": [ "2.6.1.58" ], "accessions": null } ], "citations": [ { "id": "PMID:8294935", "title": "Purification and characterization of kynurenine aminotransferase I from human brain", "journal": "J Neurochem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8294935/" }, { "id": "PMID:19338303", "title": "Structural insight into the inhibition of human kynurenine aminotransferase I/glutamine transaminase K", "journal": "J Med Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19338303/" }, { "id": "PMID:7883047", "title": "Molecular cloning and expression of a cDNA for human kidney cysteine conjugate beta-lyase", "journal": "FEBS Lett", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7883047/" }, { "id": "PMID:15364907", "title": "Crystal structure of human kynurenine aminotransferase I", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15364907/" } ], "x": 7078.666666666667, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-893596", "label": "PXLP-KYAT1 dimer transaminates L-KYN to KYNA", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:65561" ], "source_url": "https://reactome.org/content/detail/R-HSA-893596", "summary": "PXLP-KYAT1 dimer transaminates L-KYN to KYNA is a Reactome release 97 curated reaction event. The source record connects L-KYN, 2OG to KYNA, L-Glu, H2O in cytosol and cross-references RHEA:65561.", "equation": "L-KYN + 2OG → KYNA + L-Glu + H2O", "inputs": [ { "node_id": "R-ALL-29970", "reactome_id": "R-ALL-29970", "label": "L-KYN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57959", "formula": "C10H12N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-893587", "reactome_id": "R-ALL-893587", "label": "KYNA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58454", "formula": "C10H6NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-kynurenine:2-oxoglutarate transaminase activity of PXLP-KYAT1 dimer [cytosol]", "activity_id": "893577", "ec_numbers": [ "2.6.1.7" ], "accessions": null } ], "citations": [ { "id": "PMID:8294935", "title": "Purification and characterization of kynurenine aminotransferase I from human brain", "journal": "J Neurochem", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/8294935/" }, { "id": "PMID:19338303", "title": "Structural insight into the inhibition of human kynurenine aminotransferase I/glutamine transaminase K", "journal": "J Med Chem", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19338303/" }, { "id": "PMID:13069505", "title": "The transamination of kynurenine", "journal": "J Biol Chem", "year": 1953, "url": "https://pubmed.ncbi.nlm.nih.gov/13069505/" }, { "id": "PMID:7883047", "title": "Molecular cloning and expression of a cDNA for human kidney cysteine conjugate beta-lyase", "journal": "FEBS Lett", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7883047/" }, { "id": "PMID:15364907", "title": "Crystal structure of human kynurenine aminotransferase I", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15364907/" } ], "x": 8018.666666666667, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8942575", "label": "N-acetylaspartylglutamate synthase A ligates NAA, L-Glu forming NAAG", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "glutamate-glutamine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:40036" ], "source_url": "https://reactome.org/content/detail/R-HSA-8942575", "summary": "N-acetylaspartylglutamate synthase A ligates NAA, L-Glu forming NAAG is a Reactome release 97 curated reaction event. The source record connects L-Glu, ATP, NAASP to ADP, NAAG, H+ in cytosol and cross-references RHEA:40036.", "equation": "L-Glu + ATP + NAASP → ADP + NAAG + H+ + Pi", "inputs": [ { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-5693772", "reactome_id": "R-ALL-5693772", "label": "NAASP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16953", "formula": "C6H7NO5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-5693762", "reactome_id": "R-ALL-5693762", "label": "NAAG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:76931", "formula": "C11H13N2O8", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "N-acetyl-L-aspartate-L-glutamate ligase activity of RIMKLA, RIMKLB [cytosol]", "activity_id": "8942574", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:20657015", "title": "Molecular identification of N-acetylaspartylglutamate synthase and beta-citrylglutamate synthase", "journal": "J. Biol. Chem.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20657015/" } ], "x": 8018.666666666667, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8943279", "label": "GGT dimers hydrolyse GSH", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "biological-oxidations", "glutathione", "phase-two" ], "compartment": "plasma membrane · extracellular region", "compartment_ids": [ "GO:0005886", "GO:0005576" ], "rhea_ids": [ "RHEA:28808" ], "source_url": "https://reactome.org/content/detail/R-HSA-8943279", "summary": "GGT dimers hydrolyse GSH is a Reactome release 97 curated reaction event. The source record connects GSH, H2O to CysGly, L-Glu in plasma membrane · extracellular region and cross-references RHEA:28808.", "equation": "GSH + H2O → CysGly + L-Glu", "inputs": [ { "node_id": "R-ALL-1247892", "reactome_id": "R-ALL-1247892", "label": "GSH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16856", "formula": "C10H17N3O6S", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-109276", "reactome_id": "R-ALL-109276", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-1247934", "reactome_id": "R-ALL-1247934", "label": "CysGly", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:61694", "formula": "C5H10N2O3S", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-210382", "reactome_id": "R-ALL-210382", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glutathione gamma-glutamate hydrolase activity of GGT dimers [plasma membrane]", "activity_id": "8943271", "ec_numbers": [ "3.4.19.13" ], "accessions": null } ], "citations": [ { "id": "PMID:17924658", "title": "Kinetic characterization and identification of the acylation and glycosylation sites of recombinant human gamma-glutamyltranspeptidase", "journal": "Biochemistry", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17924658/" }, { "id": "PMID:8813074", "title": "Immunohistochemical detection of gamma-glutamyl transpeptidase in normal human tissue", "journal": "J. Histochem. Cytochem.", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8813074/" }, { "id": "PMID:18357469", "title": "The human gamma-glutamyltransferase gene family", "journal": "Hum Genet", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18357469/" }, { "id": "PMID:2573352", "title": "Different gamma-glutamyl transpeptidase mRNAs are expressed in human liver and kidney", "journal": "Biochem Biophys Res Commun", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2573352/" }, { "id": "PMID:19463", "title": "Human kidney gamma-glutamyl transpeptidase. Catalytic properties, subunit structure, and localization of the gamma-glutamyl binding site on the light subunit", "journal": "J Biol Chem", "year": 1977, "url": "https://pubmed.ncbi.nlm.nih.gov/19463/" }, { "id": "PMID:9080", "title": "Hydrolysis and transfer reactions catalyzed by gamma-glutamyl transpeptidase; evidence for separate substrate sites and for high affinity of L-cystine", "journal": "Biochem. Biophys. Res. Commun.", "year": 1976, "url": "https://pubmed.ncbi.nlm.nih.gov/9080/" } ], "x": 590, "y": 3556.333333333333, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8949399", "label": "CAR translocates from the cytosol to the mitochondrial intermembrane space", "schema_class": "BlackBoxEvent", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "cytosol · mitochondrial intermembrane space", "compartment_ids": [ "GO:0005829", "GO:0005758" ], "rhea_ids": [ "RHEA:34959" ], "source_url": "https://reactome.org/content/detail/R-HSA-8949399", "summary": "CAR translocates from the cytosol to the mitochondrial intermembrane space is a Reactome release 97 curated black-box event whose internal chemistry is not expanded by the source record. The source record connects CAR to CAR in cytosol · mitochondrial intermembrane space and cross-references RHEA:34959.", "equation": "CAR → CAR", "inputs": [ { "node_id": "R-ALL-30235", "reactome_id": "R-ALL-30235", "label": "CAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-549207", "reactome_id": "R-ALL-549207", "label": "CAR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [], "citations": [ { "id": "PMID:12440696", "title": "Origami in the outer membrane: the transmembrane arrangement of mitochondrial porins", "journal": "Biochem. Cell Biol.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12440696/" } ], "x": 590, "y": 2206.5, "lod": "pathway", "collapsed": true, "transport": true }, { "id": "R-HSA-8955760", "label": "PGM2L1:Mg2+ phosphorylates G6P to G1,6BP", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycolysis" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:70912" ], "source_url": "https://reactome.org/content/detail/R-HSA-8955760", "summary": "PGM2L1:Mg2+ phosphorylates G6P to G1,6BP is a Reactome release 97 curated reaction event. The source record connects G6P, 1,3BPG to G1,6BP, 3PG, H+ in cytosol and cross-references RHEA:70912.", "equation": "G6P + 1,3BPG → G1,6BP + 3PG + H+", "inputs": [ { "node_id": "R-ALL-30537", "reactome_id": "R-ALL-30537", "label": "G6P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58225", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29800", "reactome_id": "R-ALL-29800", "label": "1,3BPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57604", "formula": "C3H4O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-8955759", "reactome_id": "R-ALL-8955759", "label": "G1,6BP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58392", "formula": "C6H10O12P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29728", "reactome_id": "R-ALL-29728", "label": "3PG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58272", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "glucose-1,6-bisphosphate synthase activity of PGM2L1:Mg2+ [cytosol]", "activity_id": "8955758", "ec_numbers": [ "2.7.1.106" ], "accessions": null } ], "citations": [ { "id": "PMID:18927083", "title": "Mammalian phosphomannomutase PMM1 is the brain IMP-sensitive glucose-1,6-bisphosphatase", "journal": "J. Biol. Chem.", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18927083/" }, { "id": "PMID:17804405", "title": "Molecular identification of mammalian phosphopentomutase and glucose-1,6-bisphosphate synthase, two members of the alpha-D-phosphohexomutase family", "journal": "J Biol Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17804405/" } ], "x": 590, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8955794", "label": "PGP:Mg2+ dimer hydrolyses G3P to glycerol", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:66373" ], "source_url": "https://reactome.org/content/detail/R-HSA-8955794", "summary": "PGP:Mg2+ dimer hydrolyses G3P to glycerol is a Reactome release 97 curated reaction event. The source record connects H2O, G3P to Glycerol, Pi in cytosol and cross-references RHEA:66373.", "equation": "H2O + G3P → Glycerol + Pi", "inputs": [ { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76114", "reactome_id": "R-ALL-76114", "label": "G3P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57597", "formula": "C3H7O6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76116", "reactome_id": "R-ALL-76116", "label": "Glycerol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17754", "formula": "C3H8O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "sn-glycerol 3-phosphatase activity of PGP:Mg2+ dimer [cytosol]", "activity_id": "8955800", "ec_numbers": [ "3.1.3.21" ], "accessions": null } ], "citations": [ { "id": "PMID:35272070", "title": "Glycerol-3-phosphate phosphatase operates a glycerol shunt in pancreatic β-cells that controls insulin secretion and metabolic stress", "journal": "Mol Metab", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/35272070/" }, { "id": "PMID:36198384", "title": "Hepatic glycerol shunt and glycerol-3-phosphate phosphatase control liver metabolism and glucodetoxification under hyperglycemia", "journal": "Mol Metab", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/36198384/" }, { "id": "PMID:26755581", "title": "Identification of a mammalian glycerol-3-phosphate phosphatase: Role in metabolism and signaling in pancreatic β-cells and hepatocytes", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/26755581/" }, { "id": "PMID:39542419", "title": "Essential role of germ cell glycerol-3-phosphate phosphatase for sperm health, oxidative stress control and male fertility in mice", "journal": "Mol Metab", "year": 2024, "url": "https://pubmed.ncbi.nlm.nih.gov/39542419/" }, { "id": "PMID:24338473", "title": "Evolutionary and structural analyses of mammalian haloacid dehalogenase-type phosphatases AUM and chronophin provide insight into the basis of their different substrate specificities", "journal": "J Biol Chem", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24338473/" } ], "x": 2660, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8955844", "label": "RBKS phosphorylates ribose to R5P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13698" ], "source_url": "https://reactome.org/content/detail/R-HSA-8955844", "summary": "RBKS phosphorylates ribose to R5P is a Reactome release 97 curated reaction event. The source record connects D-ribose, ATP to R5P, ADP, H+ in cytosol and cross-references RHEA:13698.", "equation": "D-ribose + ATP → R5P + ADP + H+", "inputs": [ { "node_id": "R-ALL-8955853", "reactome_id": "R-ALL-8955853", "label": "D-ribose", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:47013", "formula": "C5H10O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-73578", "reactome_id": "R-ALL-73578", "label": "R5P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:78346", "formula": "C5H9O8P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ribokinase activity of RBKS [cytosol]", "activity_id": "8955849", "ec_numbers": [ "2.7.1.15" ], "accessions": null } ], "citations": [ { "id": "PMID:17585908", "title": "Identification and characterization of human ribokinase and comparison of its properties with E. coli ribokinase and human adenosine kinase", "journal": "FEBS Lett.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17585908/" }, { "id": "PMID:18560757", "title": "Adenosine kinase and ribokinase--the RK family of proteins", "journal": "Cell. Mol. Life Sci.", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18560757/" } ], "x": 868.6666666666667, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-8959719", "label": "SHPK phosphorylates Sedo to Sedo7P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "pentose-phosphate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23845" ], "source_url": "https://reactome.org/content/detail/R-HSA-8959719", "summary": "SHPK phosphorylates Sedo to Sedo7P is a Reactome release 97 curated reaction event. The source record connects Sedo, ATP to SH7P, ADP, H+ in cytosol and cross-references RHEA:23845.", "equation": "Sedo + ATP → SH7P + ADP + H+", "inputs": [ { "node_id": "R-ALL-8959707", "reactome_id": "R-ALL-8959707", "label": "Sedo", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16802", "formula": "C7H14O7", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29882", "reactome_id": "R-ALL-29882", "label": "SH7P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57483", "formula": "C7H13O10P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "sedoheptulokinase activity of SHPK [cytosol]", "activity_id": "8959703", "ec_numbers": [ "2.7.1.14" ], "accessions": null } ], "citations": [ { "id": "PMID:22682222", "title": "The sedoheptulose kinase CARKL directs macrophage polarization through control of glucose metabolism", "journal": "Cell Metab.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22682222/" }, { "id": "PMID:18186520", "title": "Sedoheptulokinase deficiency due to a 57-kb deletion in cystinosis patients causes urinary accumulation of sedoheptulose: elucidation of the CARKL gene", "journal": "Hum. Mutat.", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18186520/" }, { "id": "PMID:18775706", "title": "Characterization of mammalian sedoheptulokinase and mechanism of formation of erythritol in sedoheptulokinase deficiency", "journal": "FEBS Lett.", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18775706/" } ], "x": 311.33333333333337, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-901097", "label": "PXLP-KYAT3 (CCBL2) dimer transaminates L-KYN to KYNA", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "tryptophan" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:65561" ], "source_url": "https://reactome.org/content/detail/R-HSA-901097", "summary": "PXLP-KYAT3 (CCBL2) dimer transaminates L-KYN to KYNA is a Reactome release 97 curated reaction event. The source record connects L-KYN, 2OG to KYNA, L-Glu, H2O in cytosol and cross-references RHEA:65561.", "equation": "L-KYN + 2OG → KYNA + L-Glu + H2O", "inputs": [ { "node_id": "R-ALL-29970", "reactome_id": "R-ALL-29970", "label": "L-KYN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57959", "formula": "C10H12N2O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-893587", "reactome_id": "R-ALL-893587", "label": "KYNA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58454", "formula": "C10H6NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-kynurenine:2-oxoglutarate transaminase activity of CCBL2 dimer [cytosol]", "activity_id": "901096", "ec_numbers": [ "2.6.1.7" ], "accessions": null } ], "citations": [ { "id": "PMID:16376499", "title": "Characterization of kynurenine aminotransferase III, a novel member of a phylogenetically conserved KAT family", "journal": "Gene", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16376499/" } ], "x": 7461.333333333333, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9012016", "label": "FAHD1 dimer hydrolyses OA to PYR", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:15642" ], "source_url": "https://reactome.org/content/detail/R-HSA-9012016", "summary": "FAHD1 dimer hydrolyses OA to PYR is a Reactome release 97 curated reaction event. The source record connects OA, H+ to PYR, CO2 in mitochondrial matrix and cross-references RHEA:15642.", "equation": "OA + H+ → PYR + CO2", "inputs": [ { "node_id": "R-ALL-113587", "reactome_id": "R-ALL-113587", "label": "OA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113557", "reactome_id": "R-ALL-113557", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "oxaloacetate decarboxylase activity of FAHD1:Mg2+ dimer [mitochondrial matrix]", "activity_id": "9012042", "ec_numbers": [ "4.1.1.112" ], "accessions": null } ], "citations": [ { "id": "PMID:15551868", "title": "X-ray structure of fumarylacetoacetate hydrolase family member Homo sapiens FLJ36880", "journal": "Biol. Chem.", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15551868/" }, { "id": "PMID:25575590", "title": "Identification of FAH domain-containing protein 1 (FAHD1) as oxaloacetate decarboxylase", "journal": "J. Biol. Chem.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25575590/" } ], "x": 5008.666666666667, "y": 506.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9012036", "label": "ME1 tetramer decarboxylates MAL to PYR", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:18254" ], "source_url": "https://reactome.org/content/detail/R-HSA-9012036", "summary": "ME1 tetramer decarboxylates MAL to PYR is a Reactome release 97 curated reaction event. The source record connects MAL, NADP+ to PYR, CO2, NADPH in cytosol and cross-references RHEA:18254.", "equation": "MAL + NADP+ → PYR + CO2 + NADPH + H+", "inputs": [ { "node_id": "R-ALL-198498", "reactome_id": "R-ALL-198498", "label": "MAL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "malate dehydrogenase (decarboxylating) (NADP+) activity of ME1 tetramer [cytosol]", "activity_id": "9012039", "ec_numbers": [ "1.1.1.40" ], "accessions": null } ], "citations": [ { "id": "PMID:1935931", "title": "Malic enzyme in human liver. Intracellular distribution, purification and properties of cytosolic isozyme", "journal": "Eur. J. Biochem.", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1935931/" }, { "id": "PMID:14596586", "title": "Structure and function of malic enzymes, a new class of oxidative decarboxylases", "journal": "Biochemistry", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14596586/" }, { "id": "PMID:24998673", "title": "Structural characteristics of the nonallosteric human cytosolic malic enzyme", "journal": "Biochim. Biophys. Acta", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24998673/" }, { "id": "PMID:23284632", "title": "Biophysical characterization of the dimer and tetramer interface interactions of the human cytosolic malic enzyme", "journal": "PLoS ONE", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/23284632/" }, { "id": "PMID:33064660", "title": "Malic enzyme 1 (ME1) in the biology of cancer: it is not just intermediary metabolism", "journal": "J Mol Endocrinol", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/33064660/" }, { "id": "PMID:7757881", "title": "Purification and properties of cytosolic and mitochondrial malic enzyme isolated from human brain", "journal": "Int J Biochem Cell Biol", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7757881/" } ], "x": 4451.333333333333, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9012268", "label": "ME2 tetramer decarboxylates MAL to PYR", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:12654" ], "source_url": "https://reactome.org/content/detail/R-HSA-9012268", "summary": "ME2 tetramer decarboxylates MAL to PYR is a Reactome release 97 curated reaction event. The source record connects MAL, NAD+ to PYR, CO2, NADH in mitochondrial matrix and cross-references RHEA:12654.", "equation": "MAL + NAD+ → PYR + CO2 + NADH", "inputs": [ { "node_id": "R-ALL-113544", "reactome_id": "R-ALL-113544", "label": "MAL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113557", "reactome_id": "R-ALL-113557", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "malate dehydrogenase (decarboxylating) (NAD+) activity of ME2:Mg2+ tetramer [mitochondrial matrix]", "activity_id": "9012261", "ec_numbers": [ "1.1.1.39" ], "accessions": null } ], "citations": [ { "id": "PMID:1993674", "title": "Human NAD(+)-dependent mitochondrial malic enzyme. cDNA cloning, primary structure, and expression in Escherichia coli", "journal": "J. Biol. Chem.", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1993674/" }, { "id": "PMID:12962632", "title": "Crystal structures of substrate complexes of malic enzyme and insights into the catalytic mechanism", "journal": "Structure", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12962632/" }, { "id": "PMID:14596586", "title": "Structure and function of malic enzymes, a new class of oxidative decarboxylases", "journal": "Biochemistry", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14596586/" }, { "id": "PMID:23284632", "title": "Biophysical characterization of the dimer and tetramer interface interactions of the human cytosolic malic enzyme", "journal": "PLoS ONE", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/23284632/" }, { "id": "PMID:12121650", "title": "Molecular mechanism for the regulation of human mitochondrial NAD(P)+-dependent malic enzyme by ATP and fumarate", "journal": "Structure", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12121650/" }, { "id": "PMID:25202825", "title": "Human mitochondrial NAD(P)(+)-dependent malic enzyme participates in cutaneous melanoma progression and invasion", "journal": "J. Invest. Dermatol.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25202825/" } ], "x": 4730, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9012349", "label": "ME3 tetramer decarboxylates MAL to PYR", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:18254" ], "source_url": "https://reactome.org/content/detail/R-HSA-9012349", "summary": "ME3 tetramer decarboxylates MAL to PYR is a Reactome release 97 curated reaction event. The source record connects MAL, NADP+ to PYR, CO2, NADPH in mitochondrial matrix and cross-references RHEA:18254.", "equation": "MAL + NADP+ → PYR + CO2 + NADPH", "inputs": [ { "node_id": "R-ALL-113544", "reactome_id": "R-ALL-113544", "label": "MAL", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113563", "reactome_id": "R-ALL-113563", "label": "NADP+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-113557", "reactome_id": "R-ALL-113557", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113600", "reactome_id": "R-ALL-113600", "label": "NADPH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "malate dehydrogenase (decarboxylating) (NADP+) activity of ME3:Mg2+ tetramer [mitochondrial matrix]", "activity_id": "9012354", "ec_numbers": [ "1.1.1.40" ], "accessions": null } ], "citations": [ { "id": "PMID:7818469", "title": "Purification, cDNA cloning and heterologous expression of the human mitochondrial NADP(+)-dependent malic enzyme", "journal": "Biochem. J.", "year": 1994, "url": "https://pubmed.ncbi.nlm.nih.gov/7818469/" }, { "id": "PMID:14596586", "title": "Structure and function of malic enzymes, a new class of oxidative decarboxylases", "journal": "Biochemistry", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14596586/" }, { "id": "PMID:25594249", "title": "Mitochondrial malic enzyme 3 is important for insulin secretion in pancreatic β-cells", "journal": "Mol. Endocrinol.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/25594249/" }, { "id": "PMID:23284632", "title": "Biophysical characterization of the dimer and tetramer interface interactions of the human cytosolic malic enzyme", "journal": "PLoS ONE", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/23284632/" }, { "id": "PMID:36590518", "title": "Integrative structural and functional analysis of human malic enzyme 3: A potential therapeutic target for pancreatic cancer", "journal": "Heliyon", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/36590518/" }, { "id": "PMID:33572903", "title": "The Pancreatic β-Cell: The Perfect Redox System", "journal": "Antioxidants (Basel)", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/33572903/" } ], "x": 5008.666666666667, "y": 408.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9013198", "label": "TST transfers sulfur from S2O3(2-) to HCN to form HSCN", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "sulfur-amino-acids" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:16881", "RHEA:16882" ], "source_url": "https://reactome.org/content/detail/R-HSA-9013198", "summary": "TST transfers sulfur from S2O3(2-) to HCN to form HSCN is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects S2O3(2-), HCN to HSCN, SO3(2-), H+ in mitochondrial matrix and cross-references RHEA:16881, RHEA:16882.", "equation": "S2O3(2-) + HCN → HSCN + SO3(2-) + 2 H+", "inputs": [ { "node_id": "R-ALL-1614595", "reactome_id": "R-ALL-1614595", "label": "S2O3(2-)", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:33542", "formula": "HO3S2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9013199", "reactome_id": "R-ALL-9013199", "label": "HCN", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:18407", "formula": "CHN", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9013191", "reactome_id": "R-ALL-9013191", "label": "HSCN", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18022", "formula": "CNS", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-9013207", "reactome_id": "R-ALL-9013207", "label": "SO3(2-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17359", "formula": "O3S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-193465", "reactome_id": "R-ALL-193465", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "peroxisomal matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "thiosulfate-cyanide sulfurtransferase activity of TST [mitochondrial matrix]", "activity_id": "1614667", "ec_numbers": [ "2.8.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:18872649", "title": "Enzymatic conversion of cyanide to thiocyanate", "journal": "Am. J. Physiol.", "year": 1948, "url": "https://pubmed.ncbi.nlm.nih.gov/18872649/" }, { "id": "PMID:9070219", "title": "Cloning and expression of human liver rhodanese cDNA", "journal": "Biochem. Biophys. Res. Commun.", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9070219/" }, { "id": "PMID:19625201", "title": "A partial exploration of the potential energy surfaces of SCN and HSCN: implications for the enzyme-mediated detoxification of cyanide", "journal": "J. Mol. Graph. Model.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19625201/" } ], "x": 1530, "y": 2994.1111111111113, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-9014741", "label": "PXLP-K56-SRR dimer deaminates D-Ser", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:13978" ], "source_url": "https://reactome.org/content/detail/R-HSA-9014741", "summary": "PXLP-K56-SRR dimer deaminates D-Ser is a Reactome release 97 curated reaction event. The source record connects D-Ser to PYR, NH4+ in cytosol and cross-references RHEA:13978.", "equation": "D-Ser → PYR + NH4+", "inputs": [ { "node_id": "R-ALL-376186", "reactome_id": "R-ALL-376186", "label": "D-Ser", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:35247", "formula": "C3H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "D-serine ammonia-lyase activity of PXLP-K56-SRR dimer [cytosol]", "activity_id": "9014746", "ec_numbers": [ "4.3.1.18" ], "accessions": null } ], "citations": [ { "id": "PMID:24795622", "title": "Serine racemase: a key player in apoptosis and necrosis", "journal": "Front Synaptic Neurosci", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24795622/" }, { "id": "PMID:11054547", "title": "Human serine racemase: moleular cloning, genomic organization and functional analysis", "journal": "Gene", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/11054547/" }, { "id": "PMID:12393813", "title": "Cofactors of serine racemase that physiologically stimulate the synthesis of the N-methyl-D-aspartate (NMDA) receptor coagonist D-serine", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12393813/" }, { "id": "PMID:15536068", "title": "Serine racemase modulates intracellular D-serine levels through an alpha,beta-elimination activity", "journal": "J. Biol. Chem.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15536068/" }, { "id": "PMID:20106978", "title": "The structure of mammalian serine racemase: evidence for conformational changes upon inhibitor binding", "journal": "J. Biol. Chem.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20106978/" } ], "x": 311.33333333333337, "y": 2917.6666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9014766", "label": "PXLP-K56-SRR dimer isomerises L-Ser to D-Ser", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:10981" ], "source_url": "https://reactome.org/content/detail/R-HSA-9014766", "summary": "PXLP-K56-SRR dimer isomerises L-Ser to D-Ser is a Reactome release 97 curated reaction event. The source record connects L-Ser to D-Ser in cytosol and cross-references RHEA:10981.", "equation": "L-Ser → D-Ser", "inputs": [ { "node_id": "R-ALL-200736", "reactome_id": "R-ALL-200736", "label": "L-Ser", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:33384", "formula": "C3H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-376186", "reactome_id": "R-ALL-376186", "label": "D-Ser", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:35247", "formula": "C3H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "serine racemase activity of PXLP-K56-SRR dimer [cytosol]", "activity_id": "9014761", "ec_numbers": [ "5.1.1.18" ], "accessions": null } ], "citations": [ { "id": "PMID:24795622", "title": "Serine racemase: a key player in apoptosis and necrosis", "journal": "Front Synaptic Neurosci", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24795622/" }, { "id": "PMID:11054547", "title": "Human serine racemase: moleular cloning, genomic organization and functional analysis", "journal": "Gene", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/11054547/" }, { "id": "PMID:12393813", "title": "Cofactors of serine racemase that physiologically stimulate the synthesis of the N-methyl-D-aspartate (NMDA) receptor coagonist D-serine", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12393813/" }, { "id": "PMID:15536068", "title": "Serine racemase modulates intracellular D-serine levels through an alpha,beta-elimination activity", "journal": "J. Biol. Chem.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15536068/" }, { "id": "PMID:20106978", "title": "The structure of mammalian serine racemase: evidence for conformational changes upon inhibitor binding", "journal": "J. Biol. Chem.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20106978/" }, { "id": "PMID:15193426", "title": "Characterization and localization of a human serine racemase", "journal": "Brain Res. Mol. Brain Res.", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15193426/" } ], "x": 311.33333333333337, "y": 2994.1111111111113, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9018867", "label": "5-HEDH dehydrogenates 5(S)-Hp-18(S)-HpEPE to 18(S)-RvE2", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:52025" ], "source_url": "https://reactome.org/content/detail/R-HSA-9018867", "summary": "5-HEDH dehydrogenates 5(S)-Hp-18(S)-HpEPE to 18(S)-RvE2 is a Reactome release 97 curated reaction event. The source record connects 5(S)-Hp-18(S)-HEPE, hydrogen donor to 18(S)-RvE2, hydrogen acceptor, H2O in cytosol and cross-references RHEA:52025.", "equation": "5(S)-Hp-18(S)-HEPE + hydrogen donor → 18(S)-RvE2 + hydrogen acceptor + H2O", "inputs": [ { "node_id": "R-ALL-9020602", "reactome_id": "R-ALL-9020602", "label": "5(S)-Hp-18(S)-HEPE", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:132091", "formula": "C20H29O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1131953", "reactome_id": "R-ALL-1131953", "label": "hydrogen donor", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17499", "formula": "H2A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9022650", "reactome_id": "R-ALL-9022650", "label": "18(S)-RvE2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:136056", "formula": "C20H29O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1130886", "reactome_id": "R-ALL-1130886", "label": "hydrogen acceptor", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13193", "formula": "A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "NADPH dehydrogenase activity of 5-HEDH [cytosol]", "activity_id": "9022666", "ec_numbers": [ "1.6.99.1" ], "accessions": null } ], "citations": [ { "id": "PMID:17114001", "title": "Resolvin E2: identification and anti-inflammatory actions: pivotal role of human 5-lipoxygenase in resolvin E series biosynthesis", "journal": "Chem. Biol.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17114001/" }, { "id": "PMID:22450811", "title": "Resolvin E2 formation and impact in inflammation resolution", "journal": "J. Immunol.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22450811/" }, { "id": "PMID:19164464", "title": "Substrate selectivity of 5-hydroxyeicosanoid dehydrogenase and its inhibition by 5-hydroxy-Delta6-long-chain fatty acids", "journal": "J. Pharmacol. Exp. Ther.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19164464/" } ], "x": 5391.333333333333, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9018901", "label": "5-HEDH dehydrogenates 5(S)-Hp-18(R)-HEPE to 18(R)-RvE2", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:52021" ], "source_url": "https://reactome.org/content/detail/R-HSA-9018901", "summary": "5-HEDH dehydrogenates 5(S)-Hp-18(R)-HEPE to 18(R)-RvE2 is a Reactome release 97 curated reaction event. The source record connects 5(S)-Hp-18(R)-HEPE, hydrogen donor to 18(R)-RvE2, hydrogen acceptor, H2O in cytosol and cross-references RHEA:52021.", "equation": "5(S)-Hp-18(R)-HEPE + hydrogen donor → 18(R)-RvE2 + hydrogen acceptor + H2O", "inputs": [ { "node_id": "R-ALL-9023702", "reactome_id": "R-ALL-9023702", "label": "5(S)-Hp-18(R)-HEPE", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:132218", "formula": "C20H29O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1131953", "reactome_id": "R-ALL-1131953", "label": "hydrogen donor", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17499", "formula": "H2A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9023700", "reactome_id": "R-ALL-9023700", "label": "18(R)-RvE2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:133368", "formula": "C20H29O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1130886", "reactome_id": "R-ALL-1130886", "label": "hydrogen acceptor", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13193", "formula": "A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "NADPH dehydrogenase activity of 5-HEDH [cytosol]", "activity_id": "9022666", "ec_numbers": [ "1.6.99.1" ], "accessions": null } ], "citations": [ { "id": "PMID:22450811", "title": "Resolvin E2 formation and impact in inflammation resolution", "journal": "J. Immunol.", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22450811/" }, { "id": "PMID:17114001", "title": "Resolvin E2: identification and anti-inflammatory actions: pivotal role of human 5-lipoxygenase in resolvin E series biosynthesis", "journal": "Chem. Biol.", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17114001/" }, { "id": "PMID:19164464", "title": "Substrate selectivity of 5-hydroxyeicosanoid dehydrogenase and its inhibition by 5-hydroxy-Delta6-long-chain fatty acids", "journal": "J. Pharmacol. Exp. Ther.", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19164464/" } ], "x": 5670, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9020273", "label": "GPX4-2 reduces 17(S)-Hp-DHA to 17(S)-HDHA", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:76716" ], "source_url": "https://reactome.org/content/detail/R-HSA-9020273", "summary": "GPX4-2 reduces 17(S)-Hp-DHA to 17(S)-HDHA is a Reactome release 97 curated reaction event. The source record connects 17(S)-Hp-DHA, GSH to 17(S)-HDHA, GSSG, H2O in cytosol and cross-references RHEA:76716.", "equation": "17(S)-Hp-DHA + 2 GSH → 17(S)-HDHA + GSSG + H2O", "inputs": [ { "node_id": "R-ALL-9024012", "reactome_id": "R-ALL-9024012", "label": "17(S)-Hp-DHA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:133795", "formula": "C22H31O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29450", "reactome_id": "R-ALL-29450", "label": "GSH", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:57925", "formula": "C10H16N3O6S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9024815", "reactome_id": "R-ALL-9024815", "label": "17(S)-HDHA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:195403", "formula": "C22H31O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-111745", "reactome_id": "R-ALL-111745", "label": "GSSG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58297", "formula": "C20H30N6O12S2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phospholipid-hydroperoxide glutathione peroxidase activity of GPX4-2 [cytosol]", "activity_id": "9020570", "ec_numbers": [ "1.11.1.12" ], "accessions": null } ], "citations": [ { "id": "PMID:24170573", "title": "Expression and characterization of recombinant human phospholipid hydroperoxide glutathione peroxidase", "journal": "IUBMB Life", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/24170573/" }, { "id": "PMID:8605014", "title": "Expression of human phospholipid hydroperoxide glutathione peroxidase gene for protection of host cells from lipid hydroperoxide-mediated injury", "journal": "Biochem. Biophys. Res. Commun.", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/8605014/" } ], "x": 5948.666666666667, "y": 2157.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9024766", "label": "HPGD dimer oxidises RvD1 to 17(S)-oxo-RvD1 and 8-oxo-17(S)-RvD1", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:50129", "RHEA:50125" ], "source_url": "https://reactome.org/content/detail/R-HSA-9024766", "summary": "HPGD dimer oxidises RvD1 to 17(S)-oxo-RvD1 and 8-oxo-17(S)-RvD1 is a Reactome release 97 curated reaction event. The source record connects RvD1, NAD+ to 17(S)-oxo-RvD1, 8-oxo-17(S)-RvD1, NADH, H+ in cytosol and cross-references RHEA:50129, RHEA:50125.", "equation": "RvD1 + NAD+ → 17(S)-oxo-RvD1, 8-oxo-17(S)-RvD1 + NADH + H+", "inputs": [ { "node_id": "R-ALL-9024764", "reactome_id": "R-ALL-9024764", "label": "RvD1", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:132079", "formula": "C22H31O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-9024799", "reactome_id": "R-ALL-9024799", "label": "17(S)-oxo-RvD1, 8-oxo-17(S)-RvD1", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "15-hydroxyprostaglandin dehydrogenase (NAD+) activity of HPGD dimer [cytosol]", "activity_id": "2161623", "ec_numbers": [ "1.1.1.141" ], "accessions": null } ], "citations": [ { "id": "PMID:17244615", "title": "Resolvin D1 and its aspirin-triggered 17R epimer. Stereochemical assignments, anti-inflammatory properties, and enzymatic inactivation", "journal": "J. Biol. Chem.", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17244615/" } ], "x": 5948.666666666667, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9026408", "label": "PTGS2 dimer oxidises DPAn-3 to 13(R)-HDPAn-3", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:48853" ], "source_url": "https://reactome.org/content/detail/R-HSA-9026408", "summary": "PTGS2 dimer oxidises DPAn-3 to 13(R)-HDPAn-3 is a Reactome release 97 curated reaction event. The source record connects DPAn-3, O2, hydrogen donor to 13(R)-HDPAn-3, hydrogen acceptor, H2O in cytosol · endoplasmic reticulum membrane and cross-references RHEA:48853.", "equation": "DPAn-3 + O2 + hydrogen donor → 13(R)-HDPAn-3 + hydrogen acceptor + H2O", "inputs": [ { "node_id": "R-ALL-9608444", "reactome_id": "R-ALL-9608444", "label": "DPAn-3", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:77224", "formula": "C22H33O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1131953", "reactome_id": "R-ALL-1131953", "label": "hydrogen donor", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17499", "formula": "H2A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9026407", "reactome_id": "R-ALL-9026407", "label": "13(R)-HDPAn-3", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:90824", "formula": "C22H33O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1130886", "reactome_id": "R-ALL-1130886", "label": "hydrogen acceptor", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13193", "formula": "A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen of PTGS2 dimer [endoplasmic reticulum membrane]", "activity_id": "9026406", "ec_numbers": [ "1.13.11" ], "accessions": null } ], "citations": [ { "id": "PMID:26236990", "title": "Elucidation of novel 13-series resolvins that increase with atorvastatin and clear infections", "journal": "Nat. Med.", "year": 2015, "url": "https://pubmed.ncbi.nlm.nih.gov/26236990/" }, { "id": "PMID:27704804", "title": "Synthesis of 13(R)-Hydroxy-7Z,10Z,13R,14E,16Z,19Z Docosapentaenoic Acid (13R-HDPA) and Its Biosynthetic Conversion to the 13-Series Resolvins", "journal": "J. Nat. Prod.", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27704804/" } ], "x": 5391.333333333333, "y": 2060.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9026757", "label": "GGT hydrolyses MCTR1 to MCTR2", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:53513" ], "source_url": "https://reactome.org/content/detail/R-HSA-9026757", "summary": "GGT hydrolyses MCTR1 to MCTR2 is a Reactome release 97 curated reaction event. The source record connects MCTR1, H2O to MCTR2, L-Glu in cytosol and cross-references RHEA:53513.", "equation": "MCTR1 + H2O → MCTR2 + L-Glu", "inputs": [ { "node_id": "R-ALL-9026761", "reactome_id": "R-ALL-9026761", "label": "MCTR1", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:137407", "formula": "C32H45N3O9S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-9026760", "reactome_id": "R-ALL-9026760", "label": "MCTR2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:137408", "formula": "C27H39N2O6S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "glutathione gamma-glutamate hydrolase activity of GGT [cytosol]", "activity_id": "9026767", "ec_numbers": [ "3.4.19.13" ], "accessions": null } ], "citations": [ { "id": "PMID:25324525", "title": "Identification of 14-series sulfido-conjugated mediators that promote resolution of infection and organ protection", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/25324525/" }, { "id": "PMID:27791009", "title": "Maresin conjugates in tissue regeneration biosynthesis enzymes in human macrophages", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27791009/" }, { "id": "PMID:26881986", "title": "Identification and Actions of a Novel Third Maresin Conjugate in Tissue Regeneration: MCTR3", "journal": "PLoS ONE", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/26881986/" } ], "x": 5391.333333333333, "y": 2352.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9027532", "label": "PTGS2 dimer oxidises DHA to 13-HDHA", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "proresolving-mediators" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:48821" ], "source_url": "https://reactome.org/content/detail/R-HSA-9027532", "summary": "PTGS2 dimer oxidises DHA to 13-HDHA is a Reactome release 97 curated reaction event. The source record connects DHA, O2, hydrogen donor to 13-HDHA, hydrogen acceptor in cytosol · endoplasmic reticulum membrane and cross-references RHEA:48821.", "equation": "DHA + O2 + hydrogen donor → 13-HDHA + hydrogen acceptor", "inputs": [ { "node_id": "R-ALL-9024011", "reactome_id": "R-ALL-9024011", "label": "DHA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:77016", "formula": "C22H31O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29368", "reactome_id": "R-ALL-29368", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1131953", "reactome_id": "R-ALL-1131953", "label": "hydrogen donor", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17499", "formula": "H2A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9027547", "reactome_id": "R-ALL-9027547", "label": "13-HDHA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:90815", "formula": "C22H31O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-1130886", "reactome_id": "R-ALL-1130886", "label": "hydrogen acceptor", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:13193", "formula": "A", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "oxidoreductase activity, acting on single donors with incorporation of molecular oxygen of PTGS2 dimer [endoplasmic reticulum membrane]", "activity_id": "9027536", "ec_numbers": [ "1.13" ], "accessions": null } ], "citations": [ { "id": "PMID:12391014", "title": "Resolvins: a family of bioactive products of omega-3 fatty acid transformation circuits initiated by aspirin treatment that counter proinflammation signals", "journal": "J. Exp. Med.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12391014/" }, { "id": "PMID:20436486", "title": "Cyclooxygenase-2 generates anti-inflammatory mediators from omega-3 fatty acids", "journal": "Nat. Chem. Biol.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20436486/" } ], "x": 5391.333333333333, "y": 2255.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9034539", "label": "PXLP-K56-SRR dimer deaminates L-Ser", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19170" ], "source_url": "https://reactome.org/content/detail/R-HSA-9034539", "summary": "PXLP-K56-SRR dimer deaminates L-Ser is a Reactome release 97 curated reaction event. The source record connects L-Ser to PYR, NH4+ in cytosol and cross-references RHEA:19170.", "equation": "L-Ser → PYR + NH4+", "inputs": [ { "node_id": "R-ALL-200736", "reactome_id": "R-ALL-200736", "label": "L-Ser", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:33384", "formula": "C3H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "D-serine ammonia-lyase activity of PXLP-K56-SRR dimer [cytosol]", "activity_id": "9014746", "ec_numbers": [ "4.3.1.18" ], "accessions": null } ], "citations": [ { "id": "PMID:24795622", "title": "Serine racemase: a key player in apoptosis and necrosis", "journal": "Front Synaptic Neurosci", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24795622/" }, { "id": "PMID:12393813", "title": "Cofactors of serine racemase that physiologically stimulate the synthesis of the N-methyl-D-aspartate (NMDA) receptor coagonist D-serine", "journal": "Proc. Natl. Acad. Sci. U.S.A.", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12393813/" }, { "id": "PMID:15536068", "title": "Serine racemase modulates intracellular D-serine levels through an alpha,beta-elimination activity", "journal": "J. Biol. Chem.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15536068/" }, { "id": "PMID:20106978", "title": "The structure of mammalian serine racemase: evidence for conformational changes upon inhibitor binding", "journal": "J. Biol. Chem.", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20106978/" } ], "x": 590, "y": 2917.6666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-917979", "label": "ABCG2 tetramer transports heme from cytosol to extracellular region", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:19261" ], "source_url": "https://reactome.org/content/detail/R-HSA-917979", "summary": "ABCG2 tetramer transports heme from cytosol to extracellular region is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects ferroheme, ATP, H2O to heme, ADP, Pi in plasma membrane and cross-references RHEA:19261.", "equation": "ferroheme + ATP + H2O → heme + ADP + Pi + H+", "inputs": [ { "node_id": "R-ALL-71185", "reactome_id": "R-ALL-71185", "label": "ferroheme", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:60344", "formula": "C34H30FeN4O4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-917877", "reactome_id": "R-ALL-917877", "label": "heme", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:60344", "formula": "C34H30FeN4O4", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "ABC-type heme transporter activity of ABCG2 tetramer [plasma membrane]", "activity_id": "917884", "ec_numbers": [ "7.6.2.5" ], "accessions": null } ], "citations": [ { "id": "PMID:15001581", "title": "Characterization of oligomeric human half-ABC transporter ATP-binding cassette G2", "journal": "J. Biol. Chem.", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15001581/" }, { "id": "PMID:15044468", "title": "The stem cell marker Bcrp/ABCG2 enhances hypoxic cell survival through interactions with heme", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15044468/" }, { "id": "PMID:14576842", "title": "Multidrug resistance mediated by the breast cancer resistance protein BCRP (ABCG2)", "journal": "Oncogene", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14576842/" }, { "id": "PMID:12958161", "title": "The expression and functional characterization of ABCG2 in brain endothelial cells and vessels", "journal": "FASEB J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12958161/" } ], "x": 6800, "y": 2851.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-9605496", "label": "NME6 phosphorylates (d)NDPs to (d)NTPs", "schema_class": "Reaction", "domain_id": "nucleotides", "pathway_ids": [ "nucleotide-interconversion" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:27687", "RHEA:25238", "RHEA:25099", "RHEA:27675", "RHEA:27691", "RHEA:27679", "RHEA:27683", "RHEA:28583" ], "source_url": "https://reactome.org/content/detail/R-HSA-9605496", "summary": "NME6 phosphorylates (d)NDPs to (d)NTPs is a Reactome release 97 curated reaction event. The source record connects (d)NDP, ATP to (d)NTP, ADP in mitochondrial matrix and cross-references RHEA:27687, RHEA:25238, RHEA:25099, RHEA:27675, RHEA:27691, RHEA:27679, RHEA:27683, RHEA:28583.", "equation": "(d)NDP + ATP → (d)NTP + ADP", "inputs": [ { "node_id": "R-ALL-482803", "reactome_id": "R-ALL-482803", "label": "(d)NDP", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113593", "reactome_id": "R-ALL-113593", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-482807", "reactome_id": "R-ALL-482807", "label": "(d)NTP", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113581", "reactome_id": "R-ALL-113581", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "nucleoside diphosphate kinase activity of NME6 [mitochondrial matrix]", "activity_id": "9605498", "ec_numbers": [ "2.7.4.6" ], "accessions": null } ], "citations": [ { "id": "PMID:10618642", "title": "A novel human nucleoside diphosphate (NDP) kinase, Nm23-H6, localizes in mitochondria and affects cytokinesis", "journal": "J. Cell. Biochem.", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10618642/" }, { "id": "PMID:10453732", "title": "NME6: a new member of the nm23/nucleoside diphosphate kinase gene family located on human chromosome 3p21.3", "journal": "Hum. Genet.", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10453732/" } ], "x": 3600, "y": 3427.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9638125", "label": "PGM1:Mg2+ isomerises G1P to G6P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "galactose", "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23537" ], "source_url": "https://reactome.org/content/detail/R-HSA-9638125", "summary": "PGM1:Mg2+ isomerises G1P to G6P is a Reactome release 97 curated reaction event. The source record connects G1P to G6P in cytosol and cross-references RHEA:23537.", "equation": "G1P → G6P", "inputs": [ { "node_id": "R-ALL-29548", "reactome_id": "R-ALL-29548", "label": "G1P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58601", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-30537", "reactome_id": "R-ALL-30537", "label": "G6P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58225", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phosphoglucomutase activity of PGM1:Mg2+ [cytosol]", "activity_id": "9638129", "ec_numbers": [ "5.4.2.2" ], "accessions": null } ], "citations": [ { "id": "PMID:7902568", "title": "The classical human phosphoglucomutase (PGM1) isozyme polymorphism is generated by intragenic recombination", "journal": "Proc Natl Acad Sci U S A", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/7902568/" }, { "id": "PMID:4829444", "title": "A kinetic study of the isozymes determined by the three human phosphoglucomutase loci PGM1, PGM2, and PGM3", "journal": "Eur J Biochem", "year": 1974, "url": "https://pubmed.ncbi.nlm.nih.gov/4829444/" } ], "x": 1808.6666666666667, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9638127", "label": "PGM1:Mg2+ isomerises G6P to G1P", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:23538" ], "source_url": "https://reactome.org/content/detail/R-HSA-9638127", "summary": "PGM1:Mg2+ isomerises G6P to G1P is a Reactome release 97 curated reaction event. The source record connects G6P to G1P in cytosol and cross-references RHEA:23538.", "equation": "G6P → G1P", "inputs": [ { "node_id": "R-ALL-30537", "reactome_id": "R-ALL-30537", "label": "G6P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58225", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29548", "reactome_id": "R-ALL-29548", "label": "G1P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58601", "formula": "C6H11O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phosphoglucomutase activity of PGM1:Mg2+ [cytosol]", "activity_id": "9638129", "ec_numbers": [ "5.4.2.2" ], "accessions": null } ], "citations": [ { "id": "PMID:7902568", "title": "The classical human phosphoglucomutase (PGM1) isozyme polymorphism is generated by intragenic recombination", "journal": "Proc Natl Acad Sci U S A", "year": 1993, "url": "https://pubmed.ncbi.nlm.nih.gov/7902568/" }, { "id": "PMID:4829444", "title": "A kinetic study of the isozymes determined by the three human phosphoglucomutase loci PGM1, PGM2, and PGM3", "journal": "Eur J Biochem", "year": 1974, "url": "https://pubmed.ncbi.nlm.nih.gov/4829444/" } ], "x": 1251.3333333333333, "y": 887.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9661446", "label": "BMG, BDG translocates from ER lumen to cytosol", "schema_class": "BlackBoxEvent", "domain_id": "heme-oxidations", "pathway_ids": [ "porphyrins" ], "compartment": "cytosol · endoplasmic reticulum lumen · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005788", "GO:0005789" ], "rhea_ids": [ "RHEA:72795", "RHEA:72791" ], "source_url": "https://reactome.org/content/detail/R-HSA-9661446", "summary": "BMG, BDG translocates from ER lumen to cytosol is a Reactome release 97 curated black-box event whose internal chemistry is not expanded by the source record. The source record connects BMG,BDG to BMG,BDG in cytosol · endoplasmic reticulum lumen · endoplasmic reticulum membrane and cross-references RHEA:72795, RHEA:72791.", "equation": "BMG,BDG → BMG,BDG", "inputs": [ { "node_id": "R-ALL-9661424", "reactome_id": "R-ALL-9661424", "label": "BMG,BDG", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "endoplasmic reticulum lumen", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-5679042", "reactome_id": "R-ALL-5679042", "label": "BMG,BDG", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "secondary active transmembrane transporter activity of BGET [endoplasmic reticulum membrane]", "activity_id": "9663472", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:23500526", "title": "The UDP-glucuronosyltransferases: their role in drug metabolism and detoxification", "journal": "Int. J. Biochem. Cell Biol.", "year": 2013, "url": "https://pubmed.ncbi.nlm.nih.gov/23500526/" }, { "id": "PMID:24704527", "title": "Inherited disorders of bilirubin transport and conjugation: new insights into molecular mechanisms and consequences", "journal": "Gastroenterology", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24704527/" } ], "x": 6521.333333333333, "y": 2851.625, "lod": "pathway", "collapsed": true, "transport": true }, { "id": "R-HSA-9672770", "label": "SLC25A44 transports Leu, Ile and Val from cytosol to mitochondrial matrix", "schema_class": "Reaction", "domain_id": "amino-acid-carbon", "pathway_ids": [ "branched-chain" ], "compartment": "mitochondrial inner membrane", "compartment_ids": [ "GO:0005743" ], "rhea_ids": [ "RHEA:70943", "RHEA:73011", "RHEA:29703" ], "source_url": "https://reactome.org/content/detail/R-HSA-9672770", "summary": "SLC25A44 transports Leu, Ile and Val from cytosol to mitochondrial matrix is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects Leu, Ile, Val to Leu, Ile, Val in mitochondrial inner membrane and cross-references RHEA:70943, RHEA:73011, RHEA:29703.", "equation": "Leu, Ile, Val → Leu, Ile, Val", "inputs": [ { "node_id": "R-ALL-508182", "reactome_id": "R-ALL-508182", "label": "Leu, Ile, Val", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-508190", "reactome_id": "R-ALL-508190", "label": "Leu, Ile, Val", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "branched-chain amino acid transmembrane transporter activity of SLC25A44 [mitochondrial inner membrane]", "activity_id": "9672794", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:31435015", "title": "BCAA catabolism in brown fat controls energy homeostasis through SLC25A44", "journal": "Nature", "year": 2019, "url": "https://pubmed.ncbi.nlm.nih.gov/31435015/" } ], "x": 6521.333333333333, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-9695949", "label": "SPHK2 phosphorylates sphingoid", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "cytosol · endoplasmic reticulum membrane", "compartment_ids": [ "GO:0005829", "GO:0005789" ], "rhea_ids": [ "RHEA:51496" ], "source_url": "https://reactome.org/content/detail/R-HSA-9695949", "summary": "SPHK2 phosphorylates sphingoid is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects ATP, SPG to ADP, H+, S1P in cytosol · endoplasmic reticulum membrane and cross-references RHEA:51496.", "equation": "ATP + SPG → ADP + H+ + S1P", "inputs": [ { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-428682", "reactome_id": "R-ALL-428682", "label": "SPG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:84410", "formula": "C3H9NO2R", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-5279322", "reactome_id": "R-ALL-5279322", "label": "S1P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:76941", "formula": "C3H8NO5PR", "compartment": "cytoplasm", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "lipid kinase activity of SPHK2 [cytosol]", "activity_id": "9695919", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:10751414", "title": "Molecular cloning and functional characterization of a novel mammalian sphingosine kinase type 2 isoform", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10751414/" }, { "id": "PMID:16118219", "title": "SphK1 and SphK2, sphingosine kinase isoenzymes with opposing functions in sphingolipid metabolism", "journal": "J. Biol. Chem.", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16118219/" }, { "id": "PMID:28415564", "title": "Mammalian sphingosine kinase (SphK) isoenzymes and isoform expression: challenges for SphK as an oncotarget", "journal": "Oncotarget", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28415564/" }, { "id": "PMID:21864225", "title": "The compartmentalization and translocation of the sphingosine kinases: mechanisms and functions in cell signaling and sphingolipid metabolism", "journal": "Crit Rev Biochem Mol Biol", "year": 2011, "url": "https://pubmed.ncbi.nlm.nih.gov/21864225/" } ], "x": 3878.666666666667, "y": 1776.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-9709098", "label": "DHFR dimer reduces FOLA to DHF", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "folate-pterines", "water-soluble-vitamins" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:31105" ], "source_url": "https://reactome.org/content/detail/R-HSA-9709098", "summary": "DHFR dimer reduces FOLA to DHF is a Reactome release 97 curated reaction event. The source record connects FOLA, NADPH, H+ to DHF, NADP+ in cytosol and cross-references RHEA:31105.", "equation": "FOLA + NADPH + H+ → DHF + NADP+", "inputs": [ { "node_id": "R-ALL-197965", "reactome_id": "R-ALL-197965", "label": "FOLA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:62501", "formula": "C19H17N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-73604", "reactome_id": "R-ALL-73604", "label": "DHF", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57451", "formula": "C19H19N7O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "folate reductase activity of DHFR dimer [cytosol]", "activity_id": "9709122", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:6323448", "title": "The functional human dihydrofolate reductase gene", "journal": "J Biol Chem", "year": 1984, "url": "https://pubmed.ncbi.nlm.nih.gov/6323448/" }, { "id": "PMID:2248959", "title": "Crystal structures of recombinant human dihydrofolate reductase complexed with folate and 5-deazafolate", "journal": "Biochemistry", "year": 1990, "url": "https://pubmed.ncbi.nlm.nih.gov/2248959/" } ], "x": 4451.333333333333, "y": 2948.875, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9733964", "label": "SLC51A:SLC51B transports bile salts from cytosol to extracellular region", "schema_class": "Reaction", "domain_id": "lipid-mediators", "pathway_ids": [ "bile-acids", "steroids" ], "compartment": "cytosol · plasma membrane · extracellular region", "compartment_ids": [ "GO:0005829", "GO:0005886", "GO:0005576" ], "rhea_ids": [ "RHEA:71855", "RHEA:72683", "RHEA:75051", "RHEA:71859", "RHEA:71703", "RHEA:71851" ], "source_url": "https://reactome.org/content/detail/R-HSA-9733964", "summary": "SLC51A:SLC51B transports bile salts from cytosol to extracellular region is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects bile salts and acids to bile salts and acids in cytosol · plasma membrane · extracellular region and cross-references RHEA:71855, RHEA:72683, RHEA:75051, RHEA:71859, RHEA:71703, RHEA:71851.", "equation": "bile salts and acids → bile salts and acids", "inputs": [ { "node_id": "R-ALL-194156", "reactome_id": "R-ALL-194156", "label": "bile salts and acids", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "cytosol", "entity_kind": "DefinedSet", "currency": false } ], "outputs": [ { "node_id": "R-ALL-192483", "reactome_id": "R-ALL-192483", "label": "bile salts and acids", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "extracellular region", "entity_kind": "DefinedSet", "currency": false } ], "catalysts": [ { "label": "bile acid transmembrane transporter activity of SLC51A:SLC51B [plasma membrane]", "activity_id": "9733966", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:16317684", "title": "OSTalpha-OSTbeta: a major basolateral bile acid and steroid transporter in human intestinal, renal, and biliary epithelia", "journal": "Hepatology", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/16317684/" }, { "id": "PMID:17650074", "title": "Heterodimerization, trafficking and membrane topology of the two proteins, Ost alpha and Ost beta, that constitute the organic solute and steroid transporter", "journal": "Biochem J", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17650074/" } ], "x": 4730, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-977324", "label": "PSPH:Mg2+ dimer dephosphorylates O-P-Ser", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:21209" ], "source_url": "https://reactome.org/content/detail/R-HSA-977324", "summary": "PSPH:Mg2+ dimer dephosphorylates O-P-Ser is a Reactome release 97 curated reaction event. The source record connects O-P-Ser, H2O to L-Ser, Pi in cytosol and cross-references RHEA:21209.", "equation": "O-P-Ser + H2O → L-Ser + Pi", "inputs": [ { "node_id": "R-ALL-936706", "reactome_id": "R-ALL-936706", "label": "O-P-Ser", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57524", "formula": "C3H6NO6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-200736", "reactome_id": "R-ALL-200736", "label": "L-Ser", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:33384", "formula": "C3H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-phosphoserine phosphatase activity of PSPH:Mg2+ dimer [cytosol]", "activity_id": "977341", "ec_numbers": [ "3.1.3.3" ], "accessions": null } ], "citations": [ { "id": "PMID:9188776", "title": "Human L-3-phosphoserine phosphatase: sequence, expression and evidence for a phosphoenzyme intermediate", "journal": "FEBS Lett", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9188776/" } ], "x": 590, "y": 2841.222222222222, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-977333", "label": "PXLP-K200-PSAT1 dimer transfers amino group from L-Glu to 3POPA", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:14331" ], "source_url": "https://reactome.org/content/detail/R-HSA-977333", "summary": "PXLP-K200-PSAT1 dimer transfers amino group from L-Glu to 3POPA is a Reactome release 97 curated reaction event. The source record connects 3POPA, L-Glu to 2OG, O-P-Ser in cytosol and cross-references RHEA:14331.", "equation": "3POPA + L-Glu → 2OG + O-P-Ser", "inputs": [ { "node_id": "R-ALL-977329", "reactome_id": "R-ALL-977329", "label": "3POPA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:18110", "formula": "C3H2O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29404", "reactome_id": "R-ALL-29404", "label": "L-Glu", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:29985", "formula": "C5H8NO4", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-29406", "reactome_id": "R-ALL-29406", "label": "2OG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16810", "formula": "C5H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-936706", "reactome_id": "R-ALL-936706", "label": "O-P-Ser", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57524", "formula": "C3H6NO6P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "O-phospho-L-serine:2-oxoglutarate transaminase activity of PXLP-K200-PSAT1 dimer [cytosol]", "activity_id": "977342", "ec_numbers": [ "2.6.1.52" ], "accessions": null } ], "citations": [ { "id": "PMID:12633500", "title": "Characterization of human phosphoserine aminotransferase involved in the phosphorylated pathway of L-serine biosynthesis", "journal": "Biochem J", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12633500/" } ], "x": 868.6666666666667, "y": 2841.222222222222, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-977348", "label": "PHGDH tetramer dehydrogenates 3PG", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:12642" ], "source_url": "https://reactome.org/content/detail/R-HSA-977348", "summary": "PHGDH tetramer dehydrogenates 3PG is a Reactome release 97 curated reaction event. The source record connects 3PG, NAD+ to 3POPA, NADH, H+ in cytosol and cross-references RHEA:12642.", "equation": "3PG + NAD+ → 3POPA + NADH + H+", "inputs": [ { "node_id": "R-ALL-29728", "reactome_id": "R-ALL-29728", "label": "3PG", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58272", "formula": "C3H4O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-977329", "reactome_id": "R-ALL-977329", "label": "3POPA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18110", "formula": "C3H2O7P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "phosphoglycerate dehydrogenase activity of PHGDH tetramer [cytosol]", "activity_id": "977334", "ec_numbers": [ "1.1.1.95" ], "accessions": null } ], "citations": [ { "id": "PMID:19235232", "title": "Novel mutations in 3-phosphoglycerate dehydrogenase (PHGDH) are distributed throughout the protein and result in altered enzyme kinetics", "journal": "Hum Mutat", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19235232/" } ], "x": 311.33333333333337, "y": 2841.222222222222, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9773976", "label": "AKR1E2 reduces 1,5-aF to 1,5-aG", "schema_class": "Reaction", "domain_id": "carbohydrate-core", "pathway_ids": [ "glycogen" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:20667" ], "source_url": "https://reactome.org/content/detail/R-HSA-9773976", "summary": "AKR1E2 reduces 1,5-aF to 1,5-aG is a Reactome release 97 curated reaction event. The source record connects 1,5-aF, H+, NADPH to 1,5-aG, NADP+ in cytosol and cross-references RHEA:20667.", "equation": "1,5-aF + H+ + NADPH → 1,5-aG + NADP+", "inputs": [ { "node_id": "R-ALL-9773955", "reactome_id": "R-ALL-9773955", "label": "1,5-aF", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16715", "formula": "C6H10O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-9773958", "reactome_id": "R-ALL-9773958", "label": "1,5-aG", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16070", "formula": "C6H12O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "1,5-anhydro-D-fructose reductase activity of AKR1E2 [cytosol]", "activity_id": "9773965", "ec_numbers": [ "1.1.1.263" ], "accessions": null } ], "citations": [ { "id": "PMID:15118078", "title": "Characterization of htAKR, a novel gene product in the aldo-keto reductase family specifically expressed in human testis", "journal": "Mol Hum Reprod", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15118078/" }, { "id": "PMID:9022716", "title": "Hepatic production of 1,5-anhydrofructose and 1,5-anhydroglucitol in rat by the third glycogenolytic pathway", "journal": "Eur J Biochem", "year": 1996, "url": "https://pubmed.ncbi.nlm.nih.gov/9022716/" }, { "id": "PMID:12604216", "title": "Human testis specific protein: a new member of aldo-keto reductase superfamily", "journal": "Chem Biol Interact", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12604216/" }, { "id": "PMID:18323634", "title": "Mouse AKR1E1 is an ortholog of pig liver NADPH dependent 1,5-anhydro-D-fructose reductase", "journal": "Biosci Biotechnol Biochem", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18323634/" }, { "id": "PMID:9504428", "title": "Purification and some properties of a hepatic NADPH-dependent reductase that specifically acts on 1,5-anhydro-D-fructose", "journal": "J Biochem", "year": 1998, "url": "https://pubmed.ncbi.nlm.nih.gov/9504428/" }, { "id": "PMID:35534727", "title": "A glucose-like metabolite deficient in diabetes inhibits cellular entry of SARS-CoV-2", "journal": "Nat Metab", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/35534727/" } ], "x": 1530, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9843721", "label": "ABCC1,ABCG2 transport C18-S1P to extracellular region", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "cytosol · plasma membrane · extracellular region", "compartment_ids": [ "GO:0005829", "GO:0005886", "GO:0005576" ], "rhea_ids": [ "RHEA:38951" ], "source_url": "https://reactome.org/content/detail/R-HSA-9843721", "summary": "ABCC1,ABCG2 transport C18-S1P to extracellular region is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects C18-S1P, ATP, H2O to C18-S1P, ADP, H+ in cytosol · plasma membrane · extracellular region and cross-references RHEA:38951.", "equation": "C18-S1P + ATP + H2O → C18-S1P + ADP + H+ + Pi", "inputs": [ { "node_id": "R-ALL-428203", "reactome_id": "R-ALL-428203", "label": "C18-S1P", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:60119", "formula": "C18H37NO5P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113592", "reactome_id": "R-ALL-113592", "label": "ATP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:30616", "formula": "C10H12N5O13P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29356", "reactome_id": "R-ALL-29356", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-419305", "reactome_id": "R-ALL-419305", "label": "C18-S1P", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:60119", "formula": "C18H37NO5P", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29370", "reactome_id": "R-ALL-29370", "label": "ADP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:456216", "formula": "C10H12N5O10P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29372", "reactome_id": "R-ALL-29372", "label": "Pi", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:43474", "formula": "HO4P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "sphingolipid intramembrane carrier activity of ABCC1,ABCG2 [plasma membrane]", "activity_id": "9843728", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:20110355", "title": "Estradiol induces export of sphingosine 1-phosphate from breast cancer cells via ABCC1 and ABCG2", "journal": "J Biol Chem", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20110355/" }, { "id": "PMID:17050692", "title": "Role of ABCC1 in export of sphingosine-1-phosphate from mast cells", "journal": "Proc Natl Acad Sci U S A", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/17050692/" }, { "id": "PMID:19370318", "title": "Involvement of the ABC-transporter ABCC1 and the sphingosine 1-phosphate receptor subtype S1P(3) in the cytoprotection of human fibroblasts by the glucocorticoid dexamethasone", "journal": "J Mol Med (Berl)", "year": 2009, "url": "https://pubmed.ncbi.nlm.nih.gov/19370318/" } ], "x": 3878.666666666667, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-9846305", "label": "ST6GALNAC5,6 transfer Neu5Ac to GM1b", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "Golgi lumen · Golgi membrane", "compartment_ids": [ "GO:0005796", "GO:0000139" ], "rhea_ids": [ "RHEA:41968" ], "source_url": "https://reactome.org/content/detail/R-HSA-9846305", "summary": "ST6GALNAC5,6 transfer Neu5Ac to GM1b is a Reactome release 97 curated reaction event. The source record connects GM1b, CMP-Neu5Ac to GD1alpha, CMP, H+ in Golgi lumen · Golgi membrane and cross-references RHEA:41968.", "equation": "GM1b + CMP-Neu5Ac → GD1alpha + CMP + H+", "inputs": [ { "node_id": "R-ALL-9845551", "reactome_id": "R-ALL-9845551", "label": "GM1b", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:78568", "formula": "C56H95N3O31R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-727815", "reactome_id": "R-ALL-727815", "label": "CMP-Neu5Ac", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57812", "formula": "C20H29N4O16P", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9846309", "reactome_id": "R-ALL-9846309", "label": "GD1alpha", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:78569", "formula": "C67H111N4O39R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-727810", "reactome_id": "R-ALL-727810", "label": "CMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:60377", "formula": "C9H12N3O8P", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-425969", "reactome_id": "R-ALL-425969", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "alpha-N-acetylgalactosaminide alpha-2,6-sialyltransferase activity of ST6GALNAC5,6 [Golgi membrane]", "activity_id": "9846306", "ec_numbers": [ "2.4.3.3" ], "accessions": null } ], "citations": [ { "id": "PMID:12668675", "title": "Synthesis of disialyl Lewis a (Le(a)) structure in colon cancer cell lines by a sialyltransferase, ST6GalNAc VI, responsible for the synthesis of alpha-series gangliosides", "journal": "J. Biol. Chem.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12668675/" }, { "id": "PMID:10601645", "title": "A novel glycosyltransferase with a polyglutamine repeat; a new candidate for GD1alpha synthase (ST6GalNAc V)(1)", "journal": "FEBS Lett", "year": 1999, "url": "https://pubmed.ncbi.nlm.nih.gov/10601645/" } ], "x": 3321.3333333333335, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9846477", "label": "A4GALT transfers galactose to LacCer", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "Golgi membrane · Golgi lumen", "compartment_ids": [ "GO:0000139", "GO:0005796" ], "rhea_ids": [ "RHEA:11924" ], "source_url": "https://reactome.org/content/detail/R-HSA-9846477", "summary": "A4GALT transfers galactose to LacCer is a Reactome release 97 curated reaction event. The source record connects LacCer-d18, UDP-Gal to Gb3Cer, UDP, H+ in Golgi membrane · Golgi lumen and cross-references RHEA:11924.", "equation": "LacCer-d18 + UDP-Gal → Gb3Cer + UDP + H+", "inputs": [ { "node_id": "R-ALL-9844874", "reactome_id": "R-ALL-9844874", "label": "LacCer-d18", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17950", "formula": "C31H56NO13R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-735682", "reactome_id": "R-ALL-735682", "label": "UDP-Gal", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:66914", "formula": "C15H22N2O17P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-8879102", "reactome_id": "R-ALL-8879102", "label": "Gb3Cer", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:18313", "formula": "C37H66NO18R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-205687", "reactome_id": "R-ALL-205687", "label": "UDP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-425969", "reactome_id": "R-ALL-425969", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "lactosylceramide 4-alpha-galactosyltransferase activity of A4GALT [Golgi membrane]", "activity_id": "9846475", "ec_numbers": [ "2.4.1.228" ], "accessions": null } ], "citations": [ { "id": "PMID:10748143", "title": "Molecular cloning of globotriaosylceramide/CD77 synthase, a glycosyltransferase that initiates the synthesis of globo series glycosphingolipids", "journal": "J Biol Chem", "year": 2000, "url": "https://pubmed.ncbi.nlm.nih.gov/10748143/" }, { "id": "PMID:12888565", "title": "The blood group P1 synthase gene is identical to the Gb3/CD77 synthase gene. A clue to the solution of the P1/P2/p puzzle", "journal": "J Biol Chem", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12888565/" } ], "x": 3600, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9846501", "label": "B3GNT5 transfers GlcNAc to LacCer", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "sphingolipids" ], "compartment": "Golgi lumen · Golgi membrane", "compartment_ids": [ "GO:0005796", "GO:0000139" ], "rhea_ids": [ "RHEA:13905" ], "source_url": "https://reactome.org/content/detail/R-HSA-9846501", "summary": "B3GNT5 transfers GlcNAc to LacCer is a Reactome release 97 curated reaction event. The source record connects LacCer-d18, UDP-GlcNAc to Lc3Cer, UDP, H+ in Golgi lumen · Golgi membrane and cross-references RHEA:13905.", "equation": "LacCer-d18 + UDP-GlcNAc → Lc3Cer + UDP + H+", "inputs": [ { "node_id": "R-ALL-9844874", "reactome_id": "R-ALL-9844874", "label": "LacCer-d18", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17950", "formula": "C31H56NO13R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-914003", "reactome_id": "R-ALL-914003", "label": "UDP-GlcNAc", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57705", "formula": "C17H25N3O17P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9846503", "reactome_id": "R-ALL-9846503", "label": "Lc3Cer", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17103", "formula": "C39H69N2O18R", "compartment": "Golgi membrane", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-205687", "reactome_id": "R-ALL-205687", "label": "UDP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58223", "formula": "C9H11N2O12P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-425969", "reactome_id": "R-ALL-425969", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferase activity of B3GNT5 [Golgi membrane]", "activity_id": "9846493", "ec_numbers": [ "2.4.1.206" ], "accessions": null } ], "citations": [ { "id": "PMID:11283017", "title": "Molecular cloning and characterization of UDP-GlcNAc:lactosylceramide beta 1,3-N-acetylglucosaminyltransferase (beta 3Gn-T5), an essential enzyme for the expression of HNK-1 and Lewis X epitopes on glycolipids", "journal": "J Biol Chem", "year": 2001, "url": "https://pubmed.ncbi.nlm.nih.gov/11283017/" }, { "id": "PMID:20547865", "title": "Lack of lacto/neolacto-glycolipids enhances the formation of glycolipid-enriched microdomains, facilitating B cell activation", "journal": "Proc Natl Acad Sci U S A", "year": 2010, "url": "https://pubmed.ncbi.nlm.nih.gov/20547865/" } ], "x": 3878.666666666667, "y": 1873.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9854315", "label": "CSKMT methylates Citrate Synthase", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "tca" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:55544", "RHEA:55548", "RHEA:55552" ], "source_url": "https://reactome.org/content/detail/R-HSA-9854315", "summary": "CSKMT methylates Citrate Synthase is a Reactome release 97 curated reaction event. The source record connects Methylated(n) CS dimer, S-adenosyl-L-methionine to Methylated(n+1) CS dimer, H+, S-adenosyl-L-homocysteine in mitochondrial matrix and cross-references RHEA:55544, RHEA:55548, RHEA:55552.", "equation": "Methylated(n) CS dimer + 2 S-adenosyl-L-methionine → Methylated(n+1) CS dimer + 2 H+ + 2 S-adenosyl-L-homocysteine", "inputs": [ { "node_id": "R-HSA-9854325", "reactome_id": "R-HSA-9854325", "label": "Methylated(n) CS dimer", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-9854319", "reactome_id": "R-ALL-9854319", "label": "S-adenosyl-L-methionine", "role": "input", "stoichiometry": 2, "chebi_id": "CHEBI:59789", "formula": "C15H23N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-HSA-9854313", "reactome_id": "R-HSA-9854313", "label": "Methylated(n+1) CS dimer", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-9854312", "reactome_id": "R-ALL-9854312", "label": "S-adenosyl-L-homocysteine", "role": "output", "stoichiometry": 2, "chebi_id": "CHEBI:57856", "formula": "C14H20N6O5S", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "protein-lysine N-methyltransferase activity of CSKMT [mitochondrial matrix]", "activity_id": "9854316", "ec_numbers": [ "2.1.1" ], "accessions": null } ], "citations": [ { "id": "PMID:28887308", "title": "Uncovering human METTL12 as a mitochondrial methyltransferase that modulates citrate synthase activity through metabolite-sensitive lysine methylation", "journal": "J Biol Chem", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28887308/" }, { "id": "PMID:28391595", "title": "Human METTL12 is a mitochondrial methyltransferase that modifies citrate synthase", "journal": "FEBS Lett", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28391595/" }, { "id": "PMID:37533128", "title": "Methyltransferase-like proteins in cancer biology and potential therapeutic targeting", "journal": "J Hematol Oncol", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/37533128/" } ], "x": 5391.333333333333, "y": 311.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9856718", "label": "HPDL dioxygenates HPPA", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:21377" ], "source_url": "https://reactome.org/content/detail/R-HSA-9856718", "summary": "HPDL dioxygenates HPPA is a Reactome release 97 curated reaction event. The source record connects HPP, O2 to 4-HMA, CO2 in mitochondrial matrix and cross-references RHEA:21377.", "equation": "HPP + O2 → 4-HMA + CO2", "inputs": [ { "node_id": "R-ALL-9856684", "reactome_id": "R-ALL-9856684", "label": "HPP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:36242", "formula": "C9H7O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113533", "reactome_id": "R-ALL-113533", "label": "O2", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15379", "formula": "O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9856722", "reactome_id": "R-ALL-9856722", "label": "4-HMA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17210", "formula": "C8H7O4", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29376", "reactome_id": "R-ALL-29376", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "4-hydroxymandelate synthase activity of HPDL:Fe2+ [mitochondrial matrix]", "activity_id": "9856680", "ec_numbers": [ "1.13.11.46" ], "accessions": null } ], "citations": [ { "id": "PMID:37508007", "title": "Biosynthesis, Deficiency, and Supplementation of Coenzyme Q", "journal": "Antioxidants (Basel)", "year": 2023, "url": "https://pubmed.ncbi.nlm.nih.gov/37508007/" }, { "id": "PMID:34471290", "title": "The polar oxy-metabolome reveals the 4-hydroxymandelate CoQ10 synthesis pathway", "journal": "Nature", "year": 2021, "url": "https://pubmed.ncbi.nlm.nih.gov/34471290/" }, { "id": "PMID:32707086", "title": "Bi-allelic HPDL Variants Cause a Neurodegenerative Disease Ranging from Neonatal Encephalopathy to Adolescent-Onset Spastic Paraplegia", "journal": "Am J Hum Genet", "year": 2020, "url": "https://pubmed.ncbi.nlm.nih.gov/32707086/" } ], "x": 4451.333333333333, "y": 3525.125, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9856794", "label": "Unknown dehydrogenase oxidizes 4-HBz", "schema_class": "Reaction", "domain_id": "vitamins-cofactors", "pathway_ids": [ "cofactor-metabolism" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:20306" ], "source_url": "https://reactome.org/content/detail/R-HSA-9856794", "summary": "Unknown dehydrogenase oxidizes 4-HBz is a Reactome release 97 curated reaction event. The source record connects 4-Hbz, H2O, NAD+ to PHB, NADH, H+ in mitochondrial matrix and cross-references RHEA:20306.", "equation": "4-Hbz + H2O + NAD+ → PHB + NADH + H+", "inputs": [ { "node_id": "R-ALL-9856800", "reactome_id": "R-ALL-9856800", "label": "4-Hbz", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:17597", "formula": "C7H6O2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-2162213", "reactome_id": "R-ALL-2162213", "label": "PHB", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17879", "formula": "C7H5O3", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29362", "reactome_id": "R-ALL-29362", "label": "NADH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113529", "reactome_id": "R-ALL-113529", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "4-hydroxybenzaldehyde dehydrogenase (NAD+) activity of Unknown dehydrogenase [mitochondrial matrix]", "activity_id": "9856735", "ec_numbers": [ "1.2.1.64" ], "accessions": null } ], "citations": [ { "id": "PMID:27693056", "title": "Mechanistic Details of Early Steps in Coenzyme Q Biosynthesis Pathway in Yeast", "journal": "Cell Chem Biol", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27693056/" }, { "id": "PMID:27669165", "title": "Mitochondrial protein functions elucidated by multi-omic mass spectrometry profiling", "journal": "Nat Biotechnol", "year": 2016, "url": "https://pubmed.ncbi.nlm.nih.gov/27669165/" } ], "x": 4451.333333333333, "y": 3330.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9856871", "label": "MDH1 reduces OA", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "electron-transport", "malate-aspartate-shuttle" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:21434" ], "source_url": "https://reactome.org/content/detail/R-HSA-9856871", "summary": "MDH1 reduces OA is a Reactome release 97 curated reaction event. The source record connects NADH, OA, H+ to MAL, NAD+ in cytosol and cross-references RHEA:21434.", "equation": "NADH + OA + H+ → MAL + NAD+", "inputs": [ { "node_id": "R-ALL-73473", "reactome_id": "R-ALL-73473", "label": "NADH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57945", "formula": "C21H27N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-76213", "reactome_id": "R-ALL-76213", "label": "OA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-198498", "reactome_id": "R-ALL-198498", "label": "MAL", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15589", "formula": "C4H4O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29360", "reactome_id": "R-ALL-29360", "label": "NAD+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "L-malate dehydrogenase (NAD+) activity of MDH1 dimer [cytosol]", "activity_id": "198512", "ec_numbers": [ "1.1.1.37" ], "accessions": null } ], "citations": [ { "id": "PMID:2775751", "title": "Refined crystal structure of cytoplasmic malate dehydrogenase at 2.5-A resolution", "journal": "Biochemistry", "year": 1989, "url": "https://pubmed.ncbi.nlm.nih.gov/2775751/" }, { "id": "PMID:3052244", "title": "Biochemical and genetic identity of alpha-keto acid reductase and cytoplasmic malate dehydrogenase from human erythrocytes", "journal": "Ann Hum Genet", "year": 1988, "url": "https://pubmed.ncbi.nlm.nih.gov/3052244/" }, { "id": "PMID:15565635", "title": "Developmental regulation and cellular distribution of human cytosolic malate dehydrogenase (MDH1)", "journal": "J Cell Biochem", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15565635/" }, { "id": "PMID:22971926", "title": "Cytosolic malate dehydrogenase regulates senescence in human fibroblasts", "journal": "Biogerontology", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/22971926/" }, { "id": "PMID:31538237", "title": "MDH1 deficiency is a metabolic disorder of the malate-aspartate shuttle associated with early onset severe encephalopathy", "journal": "Hum Genet", "year": 2019, "url": "https://pubmed.ncbi.nlm.nih.gov/31538237/" }, { "id": "PMID:35036692", "title": "Structural Characterization of the Human Cytosolic Malate Dehydrogenase I", "journal": "ACS Omega", "year": 2022, "url": "https://pubmed.ncbi.nlm.nih.gov/35036692/" } ], "x": 4451.333333333333, "y": 790.625, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9861660", "label": "ME1 tetramer decarboxylates OA to PYR", "schema_class": "Reaction", "domain_id": "central-energy", "pathway_ids": [ "pyruvate" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:15642" ], "source_url": "https://reactome.org/content/detail/R-HSA-9861660", "summary": "ME1 tetramer decarboxylates OA to PYR is a Reactome release 97 curated reaction event. The source record connects OA, H+ to PYR, CO2 in cytosol and cross-references RHEA:15642.", "equation": "OA + H+ → PYR + CO2", "inputs": [ { "node_id": "R-ALL-76213", "reactome_id": "R-ALL-76213", "label": "OA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16452", "formula": "C4H2O5", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-113528", "reactome_id": "R-ALL-113528", "label": "CO2", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16526", "formula": "CO2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "oxaloacetate decarboxylase activity of ME1 tetramer [cytosol]", "activity_id": "9861583", "ec_numbers": [ "4.1.1.112" ], "accessions": null } ], "citations": [ { "id": "PMID:7757881", "title": "Purification and properties of cytosolic and mitochondrial malic enzyme isolated from human brain", "journal": "Int J Biochem Cell Biol", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7757881/" }, { "id": "PMID:1935931", "title": "Malic enzyme in human liver. Intracellular distribution, purification and properties of cytosolic isozyme", "journal": "Eur. J. Biochem.", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1935931/" }, { "id": "PMID:14596586", "title": "Structure and function of malic enzymes, a new class of oxidative decarboxylases", "journal": "Biochemistry", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/14596586/" }, { "id": "PMID:24998673", "title": "Structural characteristics of the nonallosteric human cytosolic malic enzyme", "journal": "Biochim. Biophys. Acta", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24998673/" }, { "id": "PMID:23284632", "title": "Biophysical characterization of the dimer and tetramer interface interactions of the human cytosolic malic enzyme", "journal": "PLoS ONE", "year": 2012, "url": "https://pubmed.ncbi.nlm.nih.gov/23284632/" } ], "x": 4451.333333333333, "y": 603.375, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9911362", "label": "CPT1B transfers PALM to CAR", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "cytosol · mitochondrial outer membrane · mitochondrial intermembrane space", "compartment_ids": [ "GO:0005829", "GO:0005741", "GO:0005758" ], "rhea_ids": [ "RHEA:12661", "RHEA:12662" ], "source_url": "https://reactome.org/content/detail/R-HSA-9911362", "summary": "CPT1B transfers PALM to CAR is a Reactome release 97 curated reaction event. The source record connects CAR, PALM-CoA to CoA-SH, L-PCAR in cytosol · mitochondrial outer membrane · mitochondrial intermembrane space and cross-references RHEA:12661, RHEA:12662.", "equation": "CAR + PALM-CoA → CoA-SH + L-PCAR", "inputs": [ { "node_id": "R-ALL-549207", "reactome_id": "R-ALL-549207", "label": "CAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-200993", "reactome_id": "R-ALL-200993", "label": "PALM-CoA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57379", "formula": "C37H62N7O17P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-76194", "reactome_id": "R-ALL-76194", "label": "CoA-SH", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57287", "formula": "C21H32N7O16P3S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-201034", "reactome_id": "R-ALL-201034", "label": "L-PCAR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:17490", "formula": "C23H45NO4", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "carnitine O-palmitoyltransferase activity of CPT1B [mitochondrial outer membrane]", "activity_id": "9911359", "ec_numbers": [ "2.3.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:9344464", "title": "Functional studies of yeast-expressed human heart muscle carnitine palmitoyltransferase I", "journal": "Arch Biochem Biophys", "year": 1997, "url": "https://pubmed.ncbi.nlm.nih.gov/9344464/" } ], "x": 868.6666666666667, "y": 2076.8333333333335, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9912889", "label": "SLC35D2 exchanges UDP-GlcA for UMP", "schema_class": "Reaction", "domain_id": "heme-oxidations", "pathway_ids": [ "biological-oxidations", "phase-two" ], "compartment": "Golgi membrane", "compartment_ids": [ "GO:0000139" ], "rhea_ids": [ "RHEA:72727" ], "source_url": "https://reactome.org/content/detail/R-HSA-9912889", "summary": "SLC35D2 exchanges UDP-GlcA for UMP is a Reactome release 97 curated reaction event. The source record connects UDP-GlcA, UMP to UDP-GlcA, UMP in Golgi membrane and cross-references RHEA:72727.", "equation": "UDP-GlcA + UMP → UDP-GlcA + UMP", "inputs": [ { "node_id": "R-ALL-158601", "reactome_id": "R-ALL-158601", "label": "UDP-GlcA", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:58052", "formula": "C15H19N2O18P2", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-735698", "reactome_id": "R-ALL-735698", "label": "UMP", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57865", "formula": "C9H11N2O9P", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-1889970", "reactome_id": "R-ALL-1889970", "label": "UDP-GlcA", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58052", "formula": "C15H19N2O18P2", "compartment": "Golgi lumen", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-109402", "reactome_id": "R-ALL-109402", "label": "UMP", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57865", "formula": "C9H11N2O9P", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "UDP-glucuronate transmembrane transporter activity of SLC35D2 [Golgi membrane]", "activity_id": "9912903", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:15607426", "title": "Identification and characterization of human Golgi nucleotide sugar transporter SLC35D2, a novel member of the SLC35 nucleotide sugar transporter family", "journal": "Genomics", "year": 2005, "url": "https://pubmed.ncbi.nlm.nih.gov/15607426/" }, { "id": "PMID:17599910", "title": "Variety of nucleotide sugar transporters with respect to the interaction with nucleoside mono- and diphosphates", "journal": "J Biol Chem", "year": 2007, "url": "https://pubmed.ncbi.nlm.nih.gov/17599910/" } ], "x": 7461.333333333333, "y": 3476.5, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9929460", "label": "SDS dimers:PXLP convert L-Ser to PYR and NH4+", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "serine" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:19170" ], "source_url": "https://reactome.org/content/detail/R-HSA-9929460", "summary": "SDS dimers:PXLP convert L-Ser to PYR and NH4+ is a Reactome release 97 curated reaction event. The source record connects L-Ser to NH4+, PYR in cytosol and cross-references RHEA:19170.", "equation": "L-Ser → NH4+ + PYR", "inputs": [ { "node_id": "R-ALL-200736", "reactome_id": "R-ALL-200736", "label": "L-Ser", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:33384", "formula": "C3H7NO3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-31633", "reactome_id": "R-ALL-31633", "label": "NH4+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28938", "formula": "H4N", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29398", "reactome_id": "R-ALL-29398", "label": "PYR", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15361", "formula": "C3H3O3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "L-serine ammonia-lyase activity of PXLP-SDS dimers [cytosol]", "activity_id": "9929461", "ec_numbers": [ "4.3.1.17" ], "accessions": null } ], "citations": [ { "id": "PMID:16580895", "title": "Enzymatic and biochemical properties of a novel human serine dehydratase isoform", "journal": "Biochim Biophys Acta", "year": 2006, "url": "https://pubmed.ncbi.nlm.nih.gov/16580895/" }, { "id": "PMID:18342636", "title": "A catalytic mechanism that explains a low catalytic activity of serine dehydratase like-1 from human cancer cells: crystal structure and site-directed mutagenesis studies", "journal": "Biochim. Biophys. Acta", "year": 2008, "url": "https://pubmed.ncbi.nlm.nih.gov/18342636/" } ], "x": 868.6666666666667, "y": 2917.6666666666665, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9931850", "label": "AKR1B1 reduces galactose to galactitol", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "galactose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:37969", "RHEA:12791" ], "source_url": "https://reactome.org/content/detail/R-HSA-9931850", "summary": "AKR1B1 reduces galactose to galactitol is a Reactome release 97 curated reaction event. The source record connects alpha-D-Gal, H+, NADPH to galactitol, NADP+ in cytosol and cross-references RHEA:37969, RHEA:12791.", "equation": "alpha-D-Gal + H+ + NADPH → galactitol + NADP+", "inputs": [ { "node_id": "R-ALL-9931851", "reactome_id": "R-ALL-9931851", "label": "alpha-D-Gal", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:28061", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-29364", "reactome_id": "R-ALL-29364", "label": "NADPH", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57783", "formula": "C21H26N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-ALL-9931848", "reactome_id": "R-ALL-9931848", "label": "galactitol", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16813", "formula": "C6H14O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-29366", "reactome_id": "R-ALL-29366", "label": "NADP+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:58349", "formula": "C21H25N7O17P3", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true } ], "catalysts": [ { "label": "aldose reductase (NADPH) activity of AKR1B1 [cytosol]", "activity_id": "196106", "ec_numbers": [ "1.1.1.21" ], "accessions": null } ], "citations": [ { "id": "PMID:7851421", "title": "Presence of a closely related subgroup in the aldo-ketoreductase family of the mouse", "journal": "Eur J Biochem", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7851421/" }, { "id": "PMID:30362099", "title": "Aldo-Keto Reductases: Multifunctional Proteins as Therapeutic Targets in Diabetes and Inflammatory Disease", "journal": "Adv Exp Med Biol", "year": 2018, "url": "https://pubmed.ncbi.nlm.nih.gov/30362099/" }, { "id": "PMID:14456181", "title": "The accumulation of dulcitol and water in rabbit lens incubated with galactose", "journal": "Biochim Biophys Acta", "year": 1962, "url": "https://pubmed.ncbi.nlm.nih.gov/14456181/" }, { "id": "PMID:4270794", "title": "Polyol accumulation in galactosemic and diabetic rats: control by an aldose reductase inhibitor", "journal": "Science", "year": 1973, "url": "https://pubmed.ncbi.nlm.nih.gov/4270794/" }, { "id": "PMID:1907924", "title": "Polyol accumulation in cultured human lens epithelial cells", "journal": "Exp Eye Res", "year": 1991, "url": "https://pubmed.ncbi.nlm.nih.gov/1907924/" }, { "id": "PMID:12570032", "title": "The in vitro effects of galactose and its derivatives on rat brain Mg2+-ATPase activity", "journal": "Pharmacol Toxicol", "year": 2002, "url": "https://pubmed.ncbi.nlm.nih.gov/12570032/" } ], "x": 3600, "y": 360.25, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9931853", "label": "GALM converts beta-D-GAL to alpha-D-GAL", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "galactose" ], "compartment": "cytosol", "compartment_ids": [ "GO:0005829" ], "rhea_ids": [ "RHEA:28677" ], "source_url": "https://reactome.org/content/detail/R-HSA-9931853", "summary": "GALM converts beta-D-GAL to alpha-D-GAL is a Reactome release 97 curated reaction event. The source record connects beta-D-Gal to alpha-D-Gal in cytosol and cross-references RHEA:28677.", "equation": "beta-D-Gal → alpha-D-Gal", "inputs": [ { "node_id": "R-ALL-9931855", "reactome_id": "R-ALL-9931855", "label": "beta-D-Gal", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:27667", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9931851", "reactome_id": "R-ALL-9931851", "label": "alpha-D-Gal", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:28061", "formula": "C6H12O6", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "aldose 1-epimerase activity of GALM [cytosol]", "activity_id": "9931854", "ec_numbers": [ "5.1.3.3" ], "accessions": null } ], "citations": [ { "id": "PMID:12753898", "title": "Identification and characterisation of human aldose 1-epimerase", "journal": "FEBS Lett.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12753898/" }, { "id": "PMID:15026423", "title": "Molecular structure of human galactose mutarotase", "journal": "J Biol Chem", "year": 2004, "url": "https://pubmed.ncbi.nlm.nih.gov/15026423/" }, { "id": "PMID:7542884", "title": "Comparison of the enzymatic activities of human galactokinase GALK1 and a related human galactokinase protein GK2", "journal": "Biochem Biophys Res Commun", "year": 1995, "url": "https://pubmed.ncbi.nlm.nih.gov/7542884/" } ], "x": 3600, "y": 554.75, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9941470", "label": "SLC26A11 imports SO4(2-) and H+ into cytosol", "schema_class": "Reaction", "domain_id": "sugar-branches-glycans", "pathway_ids": [ "glycosaminoglycans" ], "compartment": "plasma membrane", "compartment_ids": [ "GO:0005886" ], "rhea_ids": [ "RHEA:28574" ], "source_url": "https://reactome.org/content/detail/R-HSA-9941470", "summary": "SLC26A11 imports SO4(2-) and H+ into cytosol is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects H+, SO4(2-) to H+, SO4(2-) in plasma membrane and cross-references RHEA:28574.", "equation": "H+ + SO4(2-) → H+ + SO4(2-)", "inputs": [ { "node_id": "R-ALL-351626", "reactome_id": "R-ALL-351626", "label": "H+", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-427648", "reactome_id": "R-ALL-427648", "label": "SO4(2-)", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16189", "formula": "O4S", "compartment": "extracellular region", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-70106", "reactome_id": "R-ALL-70106", "label": "H+", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:15378", "formula": "H", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-174375", "reactome_id": "R-ALL-174375", "label": "SO4(2-)", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:16189", "formula": "O4S", "compartment": "cytosol", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "sulfate transmembrane transporter activity of SLC26A11 [plasma membrane]", "activity_id": "8875870", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:12626430", "title": "Molecular and functional characterization of SLC26A11, a sodium-independent sulfate transporter from high endothelial venules", "journal": "FASEB J.", "year": 2003, "url": "https://pubmed.ncbi.nlm.nih.gov/12626430/" } ], "x": 2381.3333333333335, "y": 985.125, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-9955504", "label": "SIRT5 deglutarylates CPS1", "schema_class": "Reaction", "domain_id": "nitrogen-one-carbon", "pathway_ids": [ "urea-cycle" ], "compartment": "mitochondrial matrix", "compartment_ids": [ "GO:0005759" ], "rhea_ids": [ "RHEA:47665" ], "source_url": "https://reactome.org/content/detail/R-HSA-9955504", "summary": "SIRT5 deglutarylates CPS1 is a Reactome release 97 curated reaction event. The source record connects N6-glutaryl lysine CPS1, NAD+, H2O to CPS1, 2''-O-glutaryl-ADP-D-ribose(3-), NAM in mitochondrial matrix and cross-references RHEA:47665.", "equation": "N6-glutaryl lysine CPS1 + 8 NAD+ + 8 H2O → CPS1 + 8 2''-O-glutaryl-ADP-D-ribose(3-) + 8 NAM", "inputs": [ { "node_id": "R-HSA-9955510", "reactome_id": "R-HSA-9955510", "label": "N6-glutaryl lysine CPS1", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "EntityWithAccessionedSequence", "currency": false }, { "node_id": "R-ALL-113526", "reactome_id": "R-ALL-113526", "label": "NAD+", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:57540", "formula": "C21H26N7O14P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true }, { "node_id": "R-ALL-113521", "reactome_id": "R-ALL-113521", "label": "H2O", "role": "input", "stoichiometry": 8, "chebi_id": "CHEBI:15377", "formula": "H2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": true } ], "outputs": [ { "node_id": "R-HSA-70544", "reactome_id": "R-HSA-70544", "label": "CPS1", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "EntityWithAccessionedSequence", "currency": false }, { "node_id": "R-ALL-9955515", "reactome_id": "R-ALL-9955515", "label": "2''-O-glutaryl-ADP-D-ribose(3-)", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:87829", "formula": "C20H26N5O17P2", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false }, { "node_id": "R-ALL-5688274", "reactome_id": "R-ALL-5688274", "label": "NAM", "role": "output", "stoichiometry": 8, "chebi_id": "CHEBI:17154", "formula": "C6H6N2O", "compartment": "mitochondrial matrix", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "protein-glutaryllysine deglutarylase activity of SIRT5 [mitochondrial matrix]", "activity_id": "9955442", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:24703693", "title": "Lysine glutarylation is a protein posttranslational modification regulated by SIRT5", "journal": "Cell Metab", "year": 2014, "url": "https://pubmed.ncbi.nlm.nih.gov/24703693/" } ], "x": 1808.6666666666667, "y": 3313.444444444445, "lod": "pathway", "collapsed": false, "transport": false }, { "id": "R-HSA-9956308", "label": "STARD7 transports PC from ER membrane to mitochondrial outer membrane", "schema_class": "Reaction", "domain_id": "complex-lipids", "pathway_ids": [ "phospholipids" ], "compartment": "endoplasmic reticulum membrane · mitochondrial outer membrane", "compartment_ids": [ "GO:0005789", "GO:0005741" ], "rhea_ids": [ "RHEA:38571" ], "source_url": "https://reactome.org/content/detail/R-HSA-9956308", "summary": "STARD7 transports PC from ER membrane to mitochondrial outer membrane is a Reactome release 97 curated event with participant identities in more than one compartment. The source record connects PC to PC in endoplasmic reticulum membrane · mitochondrial outer membrane and cross-references RHEA:38571.", "equation": "PC → PC", "inputs": [ { "node_id": "R-ALL-426925", "reactome_id": "R-ALL-426925", "label": "PC", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:57643", "formula": "C10H18NO8PR2", "compartment": "endoplasmic reticulum membrane", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-8873803", "reactome_id": "R-ALL-8873803", "label": "PC", "role": "output", "stoichiometry": 1, "chebi_id": "CHEBI:57643", "formula": "C10H18NO8PR2", "compartment": "mitochondrial outer membrane", "entity_kind": "SimpleEntity", "currency": false } ], "catalysts": [ { "label": "phosphatidylcholine transfer activity of STARD7 [mitochondrial outer membrane]", "activity_id": "9956301", "ec_numbers": null, "accessions": null } ], "citations": [ { "id": "PMID:28821867", "title": "Identification of the N-terminal transmembrane domain of StarD7 and its importance for mitochondrial outer membrane localization and phosphatidylcholine transfer", "journal": "Sci Rep", "year": 2017, "url": "https://pubmed.ncbi.nlm.nih.gov/28821867/" } ], "x": 2938.666666666667, "y": 1581.625, "lod": "pathway", "collapsed": false, "transport": true }, { "id": "R-HSA-9971094", "label": "SLC25A20 exports excess acyl-CAR", "schema_class": "Reaction", "domain_id": "fatty-acid-flow", "pathway_ids": [ "carnitine-shuttle", "fatty-acid-metabolism" ], "compartment": "mitochondrial inner membrane", "compartment_ids": [ "GO:0005743" ], "rhea_ids": [ "RHEA:49925" ], "source_url": "https://reactome.org/content/detail/R-HSA-9971094", "summary": "SLC25A20 exports excess acyl-CAR is a Reactome release 97 curated reaction event. The source record connects Acyl-CAR, CAR to Acyl-CAR, CAR in mitochondrial inner membrane and cross-references RHEA:49925.", "equation": "Acyl-CAR + CAR → Acyl-CAR + CAR", "inputs": [ { "node_id": "R-ALL-9971097", "reactome_id": "R-ALL-9971097", "label": "Acyl-CAR", "role": "input", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial matrix", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-549207", "reactome_id": "R-ALL-549207", "label": "CAR", "role": "input", "stoichiometry": 1, "chebi_id": "CHEBI:16347", "formula": "C7H15NO3", "compartment": "mitochondrial intermembrane space", "entity_kind": "SimpleEntity", "currency": false } ], "outputs": [ { "node_id": "R-ALL-9971099", "reactome_id": "R-ALL-9971099", "label": "Acyl-CAR", "role": "output", "stoichiometry": 1, "chebi_id": "", "formula": "", "compartment": "mitochondrial intermembrane space", "entity_kind": "DefinedSet", "currency": false }, { "node_id": "R-ALL-201023", 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"edge:R-HSA-9971094:output:0", "source": "event:R-HSA-9971094", "target": "compound:R-HSA-9971094:output:R-ALL-9971099", "event_id": "R-HSA-9971094", "role": "output", "stoichiometry": 1, "path": "", "source_id": "R-HSA-9971094", "lod": "pathway" }, { "id": "edge:R-HSA-9971094:output:1", "source": "event:R-HSA-9971094", "target": "compound:R-HSA-9971094:output:R-ALL-201023", "event_id": "R-HSA-9971094", "role": "output", "stoichiometry": 1, "path": "", "source_id": "R-HSA-9971094", "lod": "reaction" } ], "learning": [ { "id": "carbohydrate-core", "title": "Carbohydrate core", "kicker": "CARBOHYDRATES", "summary": "Routes that capture, store, release and repartition six-carbon sugars, with glycolysis as a central exchange rather than an isolated tube.", "paragraphs": [ "Carbohydrate metabolism is best read as a set of reversible exchanges interrupted by strategically directed transformations. Glucose 6-phosphate sits near storage, oxidation and biosynthetic branches, so its meaning depends on which enzyme, compartment and energetic state are in view.", "The pentose phosphate pathway is not simply an alternate route to glycolysis. Its oxidative arm changes the NADPH pool, while its non-oxidative carbon rearrangements connect sugars of different lengths to ribose and glycolytic intermediates." ], "key_ideas": [ "Storage, oxidation and biosynthesis meet at sugar phosphates.", "Carbon rearrangement and redox output are separate dimensions.", "Compartment and enzyme identity determine direction." ], "pathway_ids": [ "glycolysis", "gluconeogenesis", "pentose-phosphate", "glycogen" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-70171", "url": "https://reactome.org/content/detail/R-HSA-70171" }, { "source": "Reactome pathway", "id": "R-HSA-70263", "url": "https://reactome.org/content/detail/R-HSA-70263" }, { "source": "Reactome pathway", "id": "R-HSA-71336", "url": "https://reactome.org/content/detail/R-HSA-71336" }, { "source": "Reactome pathway", "id": "R-HSA-8982491", "url": "https://reactome.org/content/detail/R-HSA-8982491" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "sugar-branches-glycans", "title": "Sugar branches \u0026 glycans", "kicker": "SUGAR BRANCHES", "summary": "Monosaccharide entry routes and the activated-sugar chemistry used to construct, remodel and recover extracellular-matrix carbohydrates.", "paragraphs": [ "Fructose and galactose do not enter central metabolism through one universal gate. Their first reactions depend on tissue enzyme expression and create distinct phosphorylated intermediates before carbon reaches the glucose-phosphate or triose-phosphate network.", "Glycosaminoglycans are not fuel polymers. Their repeating disaccharides are assembled, modified and sulfated through activated donors in the secretory system, then degraded through ordered lysosomal steps. Missing one step can block turnover even when neighboring hydrolases remain active.", "The map keeps monosaccharide catabolism and glycan assembly in one territory to show shared activated-sugar chemistry without implying that extracellular-matrix chains feed directly into glycolysis." ], "key_ideas": [ "Different dietary sugars have distinct entry chemistry.", "Activated sugars support biosynthesis as well as catabolism.", "Lysosomal glycan turnover is ordered and compartment-specific." ], "pathway_ids": [ "fructose", "galactose", "glycosaminoglycans", "oligosaccharide-recovery" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-5652084", "url": "https://reactome.org/content/detail/R-HSA-5652084" }, { "source": "Reactome pathway", "id": "R-HSA-70370", "url": "https://reactome.org/content/detail/R-HSA-70370" }, { "source": "Reactome pathway", "id": "R-HSA-1630316", "url": "https://reactome.org/content/detail/R-HSA-1630316" }, { "source": "Reactome pathway", "id": "R-HSA-8853383", "url": "https://reactome.org/content/detail/R-HSA-8853383" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "central-energy", "title": "Central energy conversion", "kicker": "ENERGY", "summary": "The mitochondrial and cytosolic interfaces that oxidize carbon, transfer reducing equivalents and couple electron movement to ATP formation.", "paragraphs": [ "The citric acid cycle is simultaneously an oxidative sequence and an exchange hub. Intermediates can leave for biosynthesis or enter from other routes, which is why anaplerotic and cataplerotic reactions belong beside the ring rather than in a footnote.", "Respiratory electron transport and ATP formation are coupled through a membrane gradient, not through a direct chemical handoff from NADH to ATP. The compartment boundary is therefore a functional component of the system." ], "key_ideas": [ "The TCA cycle is an exchange hub.", "A membrane gradient couples electron transport to ATP synthesis.", "Displayed direction is not a measured rate." ], "pathway_ids": [ "pyruvate", "tca", "electron-transport", "chemiosmosis" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-70268", "url": "https://reactome.org/content/detail/R-HSA-70268" }, { "source": "Reactome pathway", "id": "R-HSA-71403", "url": "https://reactome.org/content/detail/R-HSA-71403" }, { "source": "Reactome pathway", "id": "R-HSA-611105", "url": "https://reactome.org/content/detail/R-HSA-611105" }, { "source": "Reactome pathway", "id": "R-HSA-163210", "url": "https://reactome.org/content/detail/R-HSA-163210" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "metabolic-integration", "title": "Metabolic integration", "kicker": "INTEGRATION", "summary": "Shuttles, exchange reactions and alternate fuels that connect otherwise separated compartments and pathway neighborhoods.", "paragraphs": [ "Metabolic integration is dominated by coupled systems. The malate–aspartate shuttle combines dehydrogenases, aminotransferases and antiporters so that cytosolic reducing equivalents influence the matrix NADH pool without NADH itself crossing the inner membrane.", "Ketone bodies package acetyl-derived carbon into water-soluble molecules. Their production and use occur in different physiological contexts and enzyme complements; a shared bloodstream does not make every tissue a net producer.", "Carbon dioxide hydration and inositol-phosphate transport illustrate two different integration problems: rapid acid–base interconversion in defined compartments, and movement of highly phosphorylated signals among cytosol, nucleus and endoplasmic reticulum." ], "key_ideas": [ "Shuttles transfer equivalents through coupled reactions.", "Ketone production and utilization are not tissue-equivalent.", "Transport events are part of pathway chemistry." ], "pathway_ids": [ "malate-aspartate-shuttle", "ketone-bodies", "carbon-dioxide", "inositol-phosphate" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-9856872", "url": "https://reactome.org/content/detail/R-HSA-9856872" }, { "source": "Reactome pathway", "id": "R-HSA-74182", "url": "https://reactome.org/content/detail/R-HSA-74182" }, { "source": "Reactome pathway", "id": "R-HSA-1475029", "url": "https://reactome.org/content/detail/R-HSA-1475029" }, { "source": "Reactome pathway", "id": "R-HSA-1483249", "url": "https://reactome.org/content/detail/R-HSA-1483249" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "fatty-acid-flow", "title": "Fatty-acid flow", "kicker": "FATTY ACIDS", "summary": "Activation, mitochondrial entry, synthesis and staged oxidation of fatty-acid carbon.", "paragraphs": [ "A fatty acid must normally be activated as an acyl-CoA before many downstream reactions. For long chains, the carnitine shuttle transfers the acyl group while CoA pools remain compartmentalized.", "Each beta-oxidation turn contains a repeated chemical motif, but chain length, unsaturation and organelle context introduce distinct supporting enzymes. A repeated visual rhythm should not erase those branches." ], "key_ideas": [ "Activation precedes most acyl chemistry.", "Carnitine transfers acyl groups across mitochondrial membranes.", "Unsaturation and chain length create real branches." ], "pathway_ids": [ "fatty-acid-metabolism", "fatty-acyl-coa", "carnitine-shuttle", "beta-oxidation" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-8978868", "url": "https://reactome.org/content/detail/R-HSA-8978868" }, { "source": "Reactome pathway", "id": "R-HSA-75105", "url": "https://reactome.org/content/detail/R-HSA-75105" }, { "source": "Reactome pathway", "id": "R-HSA-200425", "url": "https://reactome.org/content/detail/R-HSA-200425" }, { "source": "Reactome pathway", "id": "R-HSA-77289", "url": "https://reactome.org/content/detail/R-HSA-77289" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "complex-lipids", "title": "Complex lipids", "kicker": "COMPLEX LIPIDS", "summary": "Membrane, storage and signaling-lipid chemistry spanning the endoplasmic reticulum, Golgi, mitochondria, lysosome and plasma membrane.", "paragraphs": [ "Complex lipid metabolism is organized around membranes. Glycerophospholipids, sphingolipids and sterols are synthesized and remodeled on particular membrane faces, then moved by vesicles, transfer proteins or transporters. The destination membrane can be as important as the headgroup name.", "Triacylglycerol stores three fatty acyl groups on glycerol, whereas phospholipids and sphingolipids contribute to bilayer structure and signaling. Hydrolysis can connect these pools, but the enzymes and products are chemically specific.", "Cholesterol biosynthesis proceeds through isoprenoid and sterol intermediates before the final double-bond and side-chain transformations. Cholesterol then becomes a membrane component and a precursor for bile acids, steroid hormones and vitamin-D-related chemistry." ], "key_ideas": [ "Membrane location is part of lipid metabolism.", "Storage and structural lipids solve different cellular problems.", "Cholesterol connects membranes to several biosynthetic branches." ], "pathway_ids": [ "phospholipids", "sphingolipids", "cholesterol", "triglycerides" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-1483257", "url": "https://reactome.org/content/detail/R-HSA-1483257" }, { "source": "Reactome pathway", "id": "R-HSA-428157", "url": "https://reactome.org/content/detail/R-HSA-428157" }, { "source": "Reactome pathway", "id": "R-HSA-191273", "url": "https://reactome.org/content/detail/R-HSA-191273" }, { "source": "Reactome pathway", "id": "R-HSA-8979227", "url": "https://reactome.org/content/detail/R-HSA-8979227" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "lipid-mediators", "title": "Lipid mediators \u0026 sterols", "kicker": "LIPID MEDIATORS", "summary": "Sterol-derived hormones, bile acids and oxygenated polyunsaturated-fatty-acid mediators.", "paragraphs": [ "Lipid mediators are often made on demand from membrane-derived precursors. Arachidonate can be oxygenated through cyclooxygenase, lipoxygenase and cytochrome-P450 branches, producing families with different receptors and lifetimes.", "Specialized pro-resolving mediators arise through staged oxygenation of omega-3 and related polyunsaturated fatty acids. Their pathway placement describes biosynthetic chemistry; it does not by itself establish concentration, cell source or effect in a specific disease state.", "Steroid and bile-acid pathways begin with cholesterol but diverge through different organelles, tissues and conjugation steps. The map uses shared ancestry as a junction while preserving each reaction sequence." ], "key_ideas": [ "On-demand synthesis begins from defined lipid precursors.", "Oxygenase branches create distinct mediator families.", "Pathway topology alone does not establish a biological effect." ], "pathway_ids": [ "steroids", "bile-acids", "arachidonate", "proresolving-mediators" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-8957322", "url": "https://reactome.org/content/detail/R-HSA-8957322" }, { "source": "Reactome pathway", "id": "R-HSA-194068", "url": "https://reactome.org/content/detail/R-HSA-194068" }, { "source": "Reactome pathway", "id": "R-HSA-2142753", "url": "https://reactome.org/content/detail/R-HSA-2142753" }, { "source": "Reactome pathway", "id": "R-HSA-9018678", "url": "https://reactome.org/content/detail/R-HSA-9018678" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "amino-acid-carbon", "title": "Amino-acid carbon", "kicker": "AMINO ACIDS", "summary": "Amino-acid-specific transformations that connect carbon skeletons to central metabolism while preserving nitrogen handling as a separate question.", "paragraphs": [ "Amino-acid catabolism begins by redistributing or removing nitrogen, then channels the remaining carbon skeleton into a smaller set of metabolic entry points. Those products include pyruvate, acetyl-CoA, acetoacetate, 2-oxoglutarate, succinyl-CoA, fumarate and oxaloacetate.", "Branched-chain amino acids share an initial transamination and oxidative-decarboxylation logic but then diverge into chain-specific reactions. Phenylalanine and tyrosine retain aromatic chemistry much longer, while tryptophan follows the chemically diverse kynurenine route.", "The familiar labels glucogenic and ketogenic summarize possible carbon destinations; they do not specify a measured whole-body flux. Reaction-level detail is needed to see where cofactors, organelles and irreversible steps constrain each route." ], "key_ideas": [ "Nitrogen handling precedes many carbon-skeleton routes.", "Several amino acids converge on a small set of central intermediates.", "Glucogenic and ketogenic labels summarize destinations, not flux." ], "pathway_ids": [ "branched-chain", "glutamate-glutamine", "phenylalanine-tyrosine", "tryptophan" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-70895", "url": "https://reactome.org/content/detail/R-HSA-70895" }, { "source": "Reactome pathway", "id": "R-HSA-8964539", "url": "https://reactome.org/content/detail/R-HSA-8964539" }, { "source": "Reactome pathway", "id": "R-HSA-8963691", "url": "https://reactome.org/content/detail/R-HSA-8963691" }, { "source": "Reactome pathway", "id": "R-HSA-71240", "url": "https://reactome.org/content/detail/R-HSA-71240" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "nitrogen-one-carbon", "title": "Nitrogen, one-carbon \u0026 thiols", "kicker": "NITROGEN + C1", "summary": "Nitrogen disposal, one-carbon transfer, sulfur chemistry and redox-buffer synthesis at the amino-acid–nucleotide interface.", "paragraphs": [ "Carbon and nitrogen move together through amino-acid reactions but are disposed of through different networks. Transamination can redistribute amino groups without itself removing nitrogen from the body; the urea cycle supplies the dedicated disposal route.", "Tetrahydrofolate derivatives carry one-carbon units at several oxidation states. Their reactions connect serine and glycine chemistry to nucleotide construction and methyl-group metabolism." ], "key_ideas": [ "Transamination redistributes nitrogen; it does not dispose of it.", "THF carries one-carbon units at several oxidation states.", "Glutathione couples redox buffering to conjugation chemistry." ], "pathway_ids": [ "serine", "sulfur-amino-acids", "glycine-glyoxylate", "urea-cycle", "glutathione" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-977347", "url": "https://reactome.org/content/detail/R-HSA-977347" }, { "source": "Reactome pathway", "id": "R-HSA-1614635", "url": "https://reactome.org/content/detail/R-HSA-1614635" }, { "source": "Reactome pathway", "id": "R-HSA-389661", "url": "https://reactome.org/content/detail/R-HSA-389661" }, { "source": "Reactome pathway", "id": "R-HSA-70635", "url": "https://reactome.org/content/detail/R-HSA-70635" }, { "source": "Reactome pathway", "id": "R-HSA-174403", "url": "https://reactome.org/content/detail/R-HSA-174403" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "nucleotides", "title": "Nucleotide economy", "kicker": "NUCLEOTIDES", "summary": "Construction, interconversion, salvage and breakdown of purine and pyrimidine nucleotides.", "paragraphs": [ "Purine and pyrimidine nucleotides are built by different architectural strategies. Purine atoms are assembled stepwise on a ribose-phosphate scaffold, whereas a pyrimidine ring is largely constructed before attachment to ribose phosphate.", "Salvage pathways recover bases and nucleosides at lower synthetic cost, while catabolic pathways dephosphorylate, deaminate, cleave and oxidize them. The balance among synthesis, salvage and degradation depends on cell context and substrate availability.", "Nucleoside diphosphate kinases and related phosphate-transfer reactions connect nucleotide pools. The map keeps individual base identities visible rather than treating ATP as a generic synonym for every nucleoside triphosphate." ], "key_ideas": [ "De novo synthesis and salvage solve different supply problems.", "Purine and pyrimidine rings follow different construction logic.", "Nucleotide pools are continually interconverted." ], "pathway_ids": [ "nucleotide-biosynthesis", "nucleotide-salvage", "nucleotide-catabolism", "nucleotide-interconversion" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-8956320", "url": "https://reactome.org/content/detail/R-HSA-8956320" }, { "source": "Reactome pathway", "id": "R-HSA-8956321", "url": "https://reactome.org/content/detail/R-HSA-8956321" }, { "source": "Reactome pathway", "id": "R-HSA-8956319", "url": "https://reactome.org/content/detail/R-HSA-8956319" }, { "source": "Reactome pathway", "id": "R-HSA-499943", "url": "https://reactome.org/content/detail/R-HSA-499943" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "vitamins-cofactors", "title": "Vitamins \u0026 cofactors", "kicker": "COFACTORS", "summary": "Uptake, activation, transport and assembly of vitamin-derived and inorganic cofactors used throughout the network.", "paragraphs": [ "Vitamins become metabolically useful only after transport and chemical activation. The resulting cofactors participate in reaction classes—acyl transfer, redox, carboxylation, rearrangement or one-carbon transfer—rather than acting as generic energy ingredients.", "A cofactor lens exposes repeated chemistry across distant pathways. It also helps distinguish a nutrient precursor from the active molecular form that appears in an enzyme reaction." ], "key_ideas": [ "The active cofactor is not always the dietary molecule.", "Cofactors reveal recurring reaction chemistry.", "Transport and compartmental activation matter." ], "pathway_ids": [ "folate-pterines", "water-soluble-vitamins", "cofactor-metabolism", "iron-sulfur" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-196757", "url": "https://reactome.org/content/detail/R-HSA-196757" }, { "source": "Reactome pathway", "id": "R-HSA-196849", "url": "https://reactome.org/content/detail/R-HSA-196849" }, { "source": "Reactome pathway", "id": "R-HSA-8978934", "url": "https://reactome.org/content/detail/R-HSA-8978934" }, { "source": "Reactome pathway", "id": "R-HSA-1362409", "url": "https://reactome.org/content/detail/R-HSA-1362409" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "heme-oxidations", "title": "Heme \u0026 biological oxidations", "kicker": "HEME + OXIDATION", "summary": "Porphyrin synthesis and turnover alongside enzyme systems that functionalize and conjugate endogenous or environmental compounds.", "paragraphs": [ "Heme synthesis alternates between mitochondrial and cytosolic reactions, beginning with aminolevulinate formation and ending with iron insertion into protoporphyrin IX. Its geography makes transport of intermediates part of the pathway logic.", "Heme degradation opens the porphyrin ring and produces biliverdin, bilirubin and iron-handling consequences. Synthesis and breakdown are therefore connected by material identity but controlled through different enzyme systems.", "Phase-I transformations introduce or expose functional groups through oxidation, reduction or hydrolysis. Phase-II enzymes transfer polar groups such as glucuronate, sulfate or glutathione. These labels organize reaction types; they are not a guarantee that Phase I always precedes Phase II for a given compound." ], "key_ideas": [ "Heme synthesis crosses mitochondrial and cytosolic space.", "Functionalization and conjugation are reaction classes, not a mandatory sequence.", "Porphyrin turnover links ring chemistry to iron handling." ], "pathway_ids": [ "porphyrins", "biological-oxidations", "phase-one", "phase-two" ], "source_links": [ { "source": "Reactome metabolism hierarchy", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Reactome pathway", "id": "R-HSA-189445", "url": "https://reactome.org/content/detail/R-HSA-189445" }, { "source": "Reactome pathway", "id": "R-HSA-211859", "url": "https://reactome.org/content/detail/R-HSA-211859" }, { "source": "Reactome pathway", "id": "R-HSA-211945", "url": "https://reactome.org/content/detail/R-HSA-211945" }, { "source": "Reactome pathway", "id": "R-HSA-156580", "url": "https://reactome.org/content/detail/R-HSA-156580" } ], "further_links": [ { "source": "Rhea reaction knowledgebase", "id": "Rhea", "url": "https://www.rhea-db.org/" }, { "source": "ChEBI chemical ontology", "id": "ChEBI", "url": "https://www.ebi.ac.uk/chebi/" } ] }, { "id": "reading-the-field", "title": "How to read a reaction field", "kicker": "MAP LITERACY", "summary": "A metabolic map is a projection of a reaction hypergraph: compounds participate in events, and events—not decorative line crossings—create biochemical connections.", "paragraphs": [ "A conventional arrow between two metabolite names can hide additional substrates, products and cofactors. Oxalosuccinate models each reaction as its own node, then attaches every admitted input and output with a role and stoichiometric coefficient. This prevents a many-participant reaction from being mistaken for several independent pairwise conversions.", "The overview level organizes pathway territories; the pathway level exposes reaction spines and primary compounds; the reaction level reveals full participants, catalysts, compartments and citations. Moving between these levels changes visual density, not the underlying biological assertion.", "Small, frequently reused compounds can create nonsensical graph shortcuts. ATP, water, protons, nicotinamide cofactors and free CoA remain visible in reaction detail but are excluded as intermediate waypoints by the default route finder. A route therefore follows source-defined reaction direction without turning every ATP-using reaction into a neighbor.", "A pulsing route encodes ordered traversal only. Its speed is intentionally uniform and carries no information about flux, concentration, thermodynamic force or tissue abundance." ], "key_ideas": [ "Reactions are hypergraph nodes.", "Semantic zoom changes detail, not evidence.", "Currency compounds stay visible but do not create route shortcuts.", "Animated traversal is not measured flux." ], "pathway_ids": null, "source_links": [ { "source": "Reactome", "id": "R-HSA-1430728", "url": "https://reactome.org/content/detail/R-HSA-1430728" }, { "source": "Rhea", "id": "Rhea", "url": "https://www.rhea-db.org/" } ], "further_links": null }, { "id": "compartment-logic", "title": "Compartments are part of identity", "kicker": "CELLULAR GEOGRAPHY", "summary": "The same chemical name in cytosol, matrix, membrane or organelle lumen represents a different graph instance with different available reactions.", "paragraphs": [ "Cellular membranes make metabolism geographically constrained. Cytosolic NADH does not simply become matrix NADH; shuttle systems transfer reducing equivalents through coupled reactions and transport steps. Long-chain acyl groups cross the inner mitochondrial membrane through carnitine-linked chemistry rather than by moving long-chain acyl-CoA unchanged.", "Oxalosuccinate therefore keys a compound instance by both molecular identity and Reactome compartment. A line crosses a compartment boundary only when the source event places participants on different sides. Visual proximity to a membrane never creates an inferred transporter.", "Some Reactome events are black boxes: the source knows that a transition occurs but does not expand balanced internal chemistry. Those events remain labeled as collapsed objects instead of receiving an invented equation." ], "key_ideas": [ "Chemical identity plus compartment defines an instance.", "Transport requires an explicit source event.", "Shuttles transfer chemical equivalents through coupled steps.", "Black-box events remain visibly collapsed." ], "pathway_ids": [ "malate-aspartate-shuttle", "carnitine-shuttle", "inositol-phosphate" ], "source_links": [ { "source": "Reactome malate–aspartate shuttle", "id": "R-HSA-9856872", "url": "https://reactome.org/content/detail/R-HSA-9856872" }, { "source": "Reactome carnitine shuttle", "id": "R-HSA-200425", "url": "https://reactome.org/content/detail/R-HSA-200425" } ], "further_links": null }, { "id": "cofactor-chemistry", "title": "Cofactors as recurring chemical strategies", "kicker": "REACTION CHEMISTRY", "summary": "Vitamin-derived cofactors are best understood by the difficult chemistry they enable across otherwise distant pathways.", "paragraphs": [ "Thiamin diphosphate stabilizes carbon-centered intermediates in oxidative decarboxylation and carbon-transfer reactions. Flavin and nicotinamide cofactors carry electrons through different molecular mechanisms. Coenzyme A activates and transfers acyl groups, while biotin carries activated carbon dioxide in carboxylation reactions.", "Pyridoxal phosphate reorganizes amino-acid bonds through a covalent enzyme-bound intermediate. Tetrahydrofolate carries one-carbon units at several oxidation states, and cobalamin supports radical rearrangement or methyl-transfer chemistry that would be difficult by ordinary ionic mechanisms.", "The Cofactor Lens searches the admitted reaction field for these molecular forms and their synonyms. Its count describes coverage in this release—not dietary requirement, nutritional status or a claim that every cofactor-dependent human reaction is represented." ], "key_ideas": [ "Cofactors solve recurring chemical problems.", "The active cofactor differs from its dietary precursor.", "A cofactor family can connect distant pathway territories.", "Coverage counts are not nutritional measurements." ], "pathway_ids": [ "water-soluble-vitamins", "cofactor-metabolism", "folate-pterines" ], "source_links": [ { "source": "Reactome vitamins and cofactors", "id": "R-HSA-196854", "url": "https://reactome.org/content/detail/R-HSA-196854" }, { "source": "Reactome cofactor metabolism", "id": "R-HSA-8978934", "url": "https://reactome.org/content/detail/R-HSA-8978934" } ], "further_links": null }, { "id": "oxalosuccinate-focus", "title": "Oxalosuccinate: a reaction intermediate, not a free-standing pool", "kicker": "MECHANISTIC FOCUS", "summary": "The namesake compound is most useful as a lesson in scale: a net pathway arrow can conceal chemically meaningful enzyme-bound steps.", "paragraphs": [ "Mitochondrial NADP-dependent isocitrate dehydrogenase couples oxidation of isocitrate to reduction of NADP+, then decarboxylates the oxidized intermediate. Rhea represents both the overall reaction and stepwise chemistry involving charge-specific (S)-oxalosuccinate.", "The map therefore shows oxalosuccinate inside a mechanistic inset rather than as a large shared metabolite station. That visual choice keeps a transient intermediate from being mistaken for an independently measured cellular pool.", "The NAD-dependent IDH3 route and NADP-dependent IDH2 route remain distinct because their cofactors, enzyme assemblies and physiological contexts are not interchangeable even when their displayed carbon products overlap." ], "key_ideas": [ "Net reactions can conceal ordered steps.", "Charge and stereochemistry are part of chemical identity.", "Shared products do not make cofactor-specific routes equivalent." ], "pathway_ids": [ "tca" ], "source_links": [ { "source": "Reactome", "id": "R-HSA-450984", "url": "https://reactome.org/content/detail/R-HSA-450984" }, { "source": "Rhea", "id": "RHEA:19630", "url": "https://www.rhea-db.org/rhea/19630" }, { "source": "ChEBI", "id": "CHEBI:153066", "url": "https://www.ebi.ac.uk/chebi/searchId.do?chebiId=CHEBI:153066" } ], "further_links": null } ] }