2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]
HCO3- + NH4+ + 2 ATP → CAP + Pi + 2 ADP + 2 H+
- Reactome
- R-HSA-70555
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:18030
Inputs
- HCO3-CHEBI:17544, CHO3, mitochondrial matrix
- NH4+CHEBI:28938, H4N, mitochondrial matrix
- 2 ATPCHEBI:30616, C10H12N5O13P3, mitochondrial matrix
Outputs
- CAPCHEBI:58228, CH2NO5P, mitochondrial matrix
- PiCHEBI:43474, HO4P, mitochondrial matrix
- 2 ADPCHEBI:456216, C10H12N5O10P2, mitochondrial matrix
- 2 H+CHEBI:15378, H, mitochondrial matrix
Catalysed by
- carbamoyl-phosphate synthase (ammonia) activity of CPS1 [mitochondrial matrix]EC 6.3.4.16; UniProt P31327
Literature
- Human carbamylphosphate synthetase I. Stabilization, purification, and partial characterization of the enzyme from human liver. J Biol Chem, 1980
- Human carbamoylphosphate synthetase I Enzyme, 1981
- 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 Biochem J, 2009
- Molecular characterization of carbamoyl-phosphate synthetase (CPS1) deficiency using human recombinant CPS1 as a key tool Hum Mutat, 2013
- Genetic, structural and biochemical basis of carbamoyl phosphate synthetase 1 deficiency Mol Genet Metab, 2010
- CPS1: Looking at an ancient enzyme in a modern light Mol Genet Metab, 2020