Reactome | Urea cycle

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Urea cycle Show undefined attributes

authored	[InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
dbId	70635
deleted	[Deleted:507853] Deletion of instance: 70693
diseasePathways	[Pathway:R-HSA-9955698] Diseases of the urea cycle - Homo sapiens [Pathway:R-HSA-9956520] ASS1 variants cause citrullinemia - Homo sapiens [Pathway:R-HSA-9956522] OTC variants cause OTC deficiency - Homo sapiens [Pathway:R-HSA-9956553] OTC main chain variants cause OTC deficiency - Homo sapiens [Pathway:R-HSA-9956551] OTC leader sequence variants cause OTC deficiency - Homo sapiens [Pathway:R-HSA-9956508] SLC25A15 variants cause hyperornithinemia-hyperammonemia-homocitrullinemia syndrome - Homo sapiens [Pathway:R-HSA-9955542] CPS1 variants cause CPS1 deficiency - Homo sapiens [Pathway:R-HSA-9956514] ARG1 variants cause hyperargininemia - Homo sapiens [Pathway:R-HSA-9956529] ASL variants cause argininosuccinate aciduria - Homo sapiens [Pathway:R-HSA-9955693] NAGS variants cause NAGS deficiency - Homo sapiens
displayName	Urea cycle
doi	10.3180/REACT_847.1
edited	[InstanceEdit:165080] D'Eustachio, P, 0000-00-00 00:00:00
eventOf	[Pathway:R-HSA-71291] Metabolism of amino acids and derivatives - Homo sapiens
goBiologicalProcess	[GO_BiologicalProcess:20612] urea cycle
hasDiagram	true
hasEHLD	false
hasEvent	[Reaction:R-HSA-70542] glutamate + acetyl CoA => N-acetyl glutamate + CoA - Homo sapiens [Reaction:R-HSA-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Homo sapiens [Reaction:R-HSA-70560] carbamoyl phosphate + ornithine => citrulline + orthophosphate - Homo sapiens [Reaction:R-HSA-70577] ASS1 tetramer:NMRAL1 dimer:NADPH transforms L-Asp and L-Cit to ARSUA - Homo sapiens [Reaction:R-HSA-70573] argininosuccinate <=> fumarate + arginine - Homo sapiens [Reaction:R-HSA-70569] arginine + H2O => ornithine + urea [ARG1] - Homo sapiens [Reaction:R-HSA-452036] arginine + H2O => ornithine + urea [ARG2] - Homo sapiens [Reaction:R-HSA-70634] ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic) - Homo sapiens [Reaction:R-HSA-9955518] SIRT5 desuccinylates CPS1 - Homo sapiens [Reaction:R-HSA-9955504] SIRT5 deglutarylates CPS1 - Homo sapiens [BlackBoxEvent:R-HSA-9956502] OTC precursor is targeted to the mitochondrial matrix and processed - Homo sapiens [BlackBoxEvent:R-HSA-9959871] ARG1 gene expression - Homo sapiens [BlackBoxEvent:R-HSA-9959874] CPS1 gene expression - Homo sapiens [BlackBoxEvent:R-HSA-9959881] OTC gene expression - Homo sapiens [Reaction:R-HSA-9959857] TP53 binds ARG1 gene - Homo sapiens [Reaction:R-HSA-9959860] TP53 binds CPS1 gene - Homo sapiens [Reaction:R-HSA-9959863] TP53 binds OTC gene - Homo sapiens
isInDisease	false
isInferred	false
lastUpdatedDate	2016-03-24
literatureReference	[Book:70549] Urea cycle enzymes 0079130356 Scriver, CR The Metabolic and Molecular Bases of Inherited Disease, 8th ed
maxDepth	2
modified	[InstanceEdit:11108703] Weiser, Joel, 2026-03-16
name	Urea cycle Urea synthesis
orthologousEvent	[Pathway:R-MMU-70635] Urea cycle - Mus musculus [Pathway:R-CFA-70635] Urea cycle - Canis familiaris [Pathway:R-BTA-70635] Urea cycle - Bos taurus [Pathway:R-SSC-70635] Urea cycle - Sus scrofa [Pathway:R-DRE-70635] Urea cycle - Danio rerio [Pathway:R-XTR-70635] Urea cycle - Xenopus tropicalis [Pathway:R-GGA-70635] Urea cycle - Gallus gallus [Pathway:R-DME-70635] Urea cycle - Drosophila melanogaster [Pathway:R-CEL-70635] Urea cycle - Caenorhabditis elegans [Pathway:R-DDI-70635] Urea cycle - Dictyostelium discoideum [Pathway:R-SPO-70635] Urea cycle - Schizosaccharomyces pombe [Pathway:R-SCE-70635] Urea cycle - Saccharomyces cerevisiae [Pathway:R-PFA-70635] Urea cycle - Plasmodium falciparum [Pathway:R-RNO-70635] Urea cycle - Rattus norvegicus
previousReviewStatus	[ReviewStatus:9821384] two stars
releaseDate	2003-03-31
releaseStatus	UPDATED
reviewStatus	[ReviewStatus:9821385] four stars
revised	[InstanceEdit:452018] D'Eustachio, P, 2010-01-14
schemaClass	Pathway
species	[Species:48887] Homo sapiens
stId	R-HSA-70635
summation	[Summation:70637] The urea cycle yields urea, the major form in which excess n...
updateTrackers	[UpdateTracker:9791746] Update Tracker - [Pathway:70635] Urea cycle - v26:[updateContainedRLE] [UpdateTracker:9783547] Update Tracker - [Pathway:70635] Urea cycle - v56:[updateContainedRLE] [UpdateTracker:9790879] Update Tracker - [Pathway:70635] Urea cycle - v32:[addHasEvent, modifyText, updateContainedRLE] [UpdateTracker:9971870] Update Tracker - [Pathway:70635] Urea cycle - v95:[removeHasEvent] [UpdateTracker:9789644] Update Tracker - [Pathway:70635] Urea cycle - v37:[updateContainedRLE]

Referrals

(hasEvent)	[Pathway:R-HSA-71291] Metabolism of amino acids and derivatives
(normalPathway)	[Pathway:R-HSA-9955698] Diseases of the urea cycle [Pathway:R-HSA-9956520] ASS1 variants cause citrullinemia [Pathway:R-HSA-9956522] OTC variants cause OTC deficiency [Pathway:R-HSA-9956553] OTC main chain variants cause OTC deficiency [Pathway:R-HSA-9956551] OTC leader sequence variants cause OTC deficiency [Pathway:R-HSA-9956508] SLC25A15 variants cause hyperornithinemia-hyperammonemia-homocitrullinemia syndrome [Pathway:R-HSA-9955542] CPS1 variants cause CPS1 deficiency [Pathway:R-HSA-9956514] ARG1 variants cause hyperargininemia [Pathway:R-HSA-9956529] ASL variants cause argininosuccinate aciduria [Pathway:R-HSA-9955693] NAGS variants cause NAGS deficiency
(replacementInstances)	[Deleted:507853] Deletion of instance: 70693
(updatedInstance)	[UpdateTracker:9791746] Update Tracker - [Pathway:70635] Urea cycle - v26:[updateContainedRLE] [UpdateTracker:9783547] Update Tracker - [Pathway:70635] Urea cycle - v56:[updateContainedRLE] [UpdateTracker:9790879] Update Tracker - [Pathway:70635] Urea cycle - v32:[addHasEvent, modifyText, updateContainedRLE] [UpdateTracker:9971870] Update Tracker - [Pathway:70635] Urea cycle - v95:[removeHasEvent] [UpdateTracker:9789644] Update Tracker - [Pathway:70635] Urea cycle - v37:[updateContainedRLE]

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