Reactome | 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]

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2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] Show undefined attributes

authored	[InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
catalystActivity	[CatalystActivity:70552] carbamoyl-phosphate synthase (ammonia) activity of CPS1 [mitochondrial matrix]
catalystActivityReference	[CatalystActivityReference:9643832] carbamoyl-phosphate synthase (ammonia) activity of CPS1 [mitochondrial matrix] Human carbamylphosphate synthetase I. Stabilization, purification, and partial characterization of the enzyme from human liver
category	transition
compartment	[Compartment:5460] mitochondrial matrix
crossReference	[DatabaseIdentifier:12667671] RHEA:18030
dbId	70555
diseaseReaction	[FailedReaction:R-HSA-9955543] CPS1 variants don't synthesize carbamoyl phosphate - Homo sapiens
displayName	2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]
edited	[InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
eventOf	[Pathway:R-HSA-70635] Urea cycle - Homo sapiens
followingEvent	[Reaction:R-HSA-70560] carbamoyl phosphate + ornithine => citrulline + orthophosphate - Homo sapiens
input	2 × [SimpleEntity:R-ALL-113593] ATP [mitochondrial matrix] [SimpleEntity:R-ALL-29896] HCO3- [mitochondrial matrix] [SimpleEntity:R-ALL-113561] NH4+ [mitochondrial matrix]
isInDisease	false
isInferred	false
literatureReference	[LiteratureReference:70545] Human carbamylphosphate synthetase I. Stabilization, purification, and partial characterization of the enzyme from human liver [LiteratureReference:9955013] Human carbamoylphosphate synthetase I [LiteratureReference:9955103] 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 [LiteratureReference:9955059] Molecular characterization of carbamoyl-phosphate synthetase (CPS1) deficiency using human recombinant CPS1 as a key tool [LiteratureReference:9955001] Genetic, structural and biochemical basis of carbamoyl phosphate synthetase 1 deficiency [LiteratureReference:9954948] CPS1: Looking at an ancient enzyme in a modern light [LiteratureReference:9954988] Expression of nuclear genes encoding the urea cycle enzymes, carbamoyl-phosphate synthetase I and ornithine carbamoyl transferase, in rat liver and intestinal mucosa [LiteratureReference:9955034] Mitochondrial carbamoyl phosphate synthetase activity in the absence of N-acetyl-L-glutamate. Mechanism of activation by this cofactor [LiteratureReference:9955106] Structure of human carbamoyl phosphate synthetase: deciphering the on/off switch of human ureagenesis [LiteratureReference:9954985] Congenital hyperammonemia. Association with hyperglycinemia and decreased levels of carbamyl phosphate synthetase [LiteratureReference:9955129] Unraveling the therapeutic potential of carbamoyl phosphate synthetase 1 (CPS1) in human diseases
maxDepth	1
modified	[InstanceEdit:11080360] Weiser, Joel, 2026-03-16
name	2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate
orthologousEvent	[Reaction:R-SCE-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Saccharomyces cerevisiae [Reaction:R-SPO-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Schizosaccharomyces pombe [Reaction:R-GGA-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Gallus gallus [Reaction:R-XTR-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Xenopus tropicalis [Reaction:R-SSC-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Sus scrofa [Reaction:R-BTA-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Bos taurus [Reaction:R-CFA-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Canis familiaris [Reaction:R-RNO-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Rattus norvegicus [Reaction:R-MMU-70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - Mus musculus
output	2 × [SimpleEntity:R-ALL-113581] ADP [mitochondrial matrix] [SimpleEntity:R-ALL-29676] CAP [mitochondrial matrix] [SimpleEntity:R-ALL-113548] Pi [mitochondrial matrix]
precedingEvent	[Reaction:R-HSA-70542] glutamate + acetyl CoA => N-acetyl glutamate + CoA - Homo sapiens [Reaction:R-HSA-73591] conversion of 3-ureidopropionate to beta-alanine - Homo sapiens [Reaction:R-HSA-9955518] SIRT5 desuccinylates CPS1 - Homo sapiens [Reaction:R-HSA-9955504] SIRT5 deglutarylates CPS1 - Homo sapiens
regulatedBy	[PositiveRegulation:R-HSA-70556] Positive regulation by 'NAcGlu [mitochondrial matrix]'
releaseDate	2004-07-06
reviewStatus	[ReviewStatus:9821382] five stars
revised	[InstanceEdit:452018] D'Eustachio, P, 2010-01-14
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-70555
summation	[Summation:9955529] Carbamoyl-phosphate synthetase 1 (CPS1) is a mitochondrial e...
updateTrackers	[UpdateTracker:9791106] Update Tracker - [Reaction:70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - v32:[removeLiteratureReference] [UpdateTracker:9964726] Update Tracker - [Reaction:70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - v94:[add_removeSummation, removeLiteratureReference]

Referrals

(updatedInstance)	[UpdateTracker:9791106] Update Tracker - [Reaction:70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - v32:[removeLiteratureReference] [UpdateTracker:9964726] Update Tracker - [Reaction:70555] 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial] - v94:[add_removeSummation, removeLiteratureReference]
(normalReaction)	[FailedReaction:R-HSA-9955543] CPS1 variants don't synthesize carbamoyl phosphate
(hasEvent)	[Pathway:R-HSA-70635] Urea cycle
(precedingEvent)	[Reaction:R-HSA-70560] carbamoyl phosphate + ornithine => citrulline + orthophosphate

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