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Details on Person 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]

Class:IdReaction:70555
_displayName2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]
_doReleaseTRUE
_timestamp2026-05-07 22:30:37
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
compartment[Compartment:5460] mitochondrial matrix
crossReference[DatabaseIdentifier:9988058] RHEA:18030
edited[InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
input[SimpleEntity:29896] HCO3- [mitochondrial matrix]
[SimpleEntity:113561] NH4+ [mitochondrial matrix]
[SimpleEntity:113593] ATP [mitochondrial matrix]
[SimpleEntity:113593] ATP [mitochondrial matrix]
internalReviewed[InstanceEdit:9988070] D'Eustachio, Peter, 2026-05-01
[InstanceEdit:9988524] D'Eustachio, Peter, 2026-05-07
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
modified[InstanceEdit:74362] D'Eustachio, P, 2003-07-18 15:39:00
[InstanceEdit:74479] D'Eustachio, P, 2003-07-22 16:21:00
[InstanceEdit:113517] Schmidt, EE, 2004-06-09 05:19:12
[InstanceEdit:163376] Vastrik, I, 2005-04-29 06:26:51
[InstanceEdit:452030] D'Eustachio, P, 2010-01-14
[InstanceEdit:580604] D'Eustachio, P, 2010-04-11
[InstanceEdit:1080818] D'Eustachio, P, 2010-11-27
[InstanceEdit:8946993] Shorser, Solomon, 2016-11-07
[InstanceEdit:9606200] Wu, G, 2018-04-19
[InstanceEdit:9643047] Wu, G, 2019-04-04
[InstanceEdit:9830342] Matthews, Lisa, 2023-03-08
[InstanceEdit:9955530] Rothfels, Karen, 2025-06-10
[InstanceEdit:9988059] D'Eustachio, Peter, 2026-05-01
[InstanceEdit:9988069] D'Eustachio, Peter, 2026-05-01
[InstanceEdit:9988428] D'Eustachio, Peter, 2026-05-07
[InstanceEdit:9988525] D'Eustachio, Peter, 2026-05-07
[InstanceEdit:9988528] D'Eustachio, Peter, 2026-05-07
name2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]
hydrogencarbonate + NH4(+) + 2 ATP => carbamoyl phosphate + 2 ADP + phosphate + 2 H(+)
output[SimpleEntity:29676] CAP [mitochondrial matrix]
[SimpleEntity:113548] Pi [mitochondrial matrix]
[SimpleEntity:113581] ADP [mitochondrial matrix]
[SimpleEntity:113581] ADP [mitochondrial matrix]
[SimpleEntity:113529] H+ [mitochondrial matrix]
[SimpleEntity:113529] H+ [mitochondrial matrix]
precedingEvent
previousReviewStatus[ReviewStatus:9821385] four stars
regulatedBy[PositiveRegulation:70556] Positive regulation by 'NAcGlu [mitochondrial matrix]'
releaseDate2004-07-06
reviewed[InstanceEdit:9988529] Hill, David P, 2026-05-07
reviewStatus[ReviewStatus:9821382] five stars
revised[InstanceEdit:452018] D'Eustachio, P, 2010-01-14
species[Species:48887] Homo sapiens
stableIdentifier[StableIdentifier:361300] R-HSA-70555.6
structureModified[InstanceEdit:9988059] D'Eustachio, Peter, 2026-05-01
summation[Summation:9955529] Carbamoyl-phosphate synthetase 1 (CPS1) is a mitochondrial e...
(hasEvent)[Pathway:70635] Urea cycle [Homo sapiens]
(normalReaction)[FailedReaction:9955543] CPS1 variants don't synthesize carbamoyl phosphate [Homo sapiens]
(precedingEvent)[Reaction:70560] carbamoyl phosphate + ornithine => citrulline + orthophosphate [Homo sapiens]
(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]
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