Carbamoyl phosphate synthase I

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carbamoyl-phosphate synthetase 1, mitochondrial
Identifiers
Symbol	CPS1
Entrez	1373
HUGO	2323
OMIM	608307
RefSeq	NM_001875
UniProt	P31327
Other data
EC number	6.3.4.16
Locus	Chr. 2 p

Carbamoyl phosphate synthase I is the enzyme responsible for catalyzing the reactions which produce Carbamoyl phosphate in the mitochondria (as opposed to type 2, which functions in the cytosol).

In the urea cycle, it takes part in this reaction:

2 ATP + HCO₃^- + NH₃ → carbamoyl phosphate + 2 ADP + P_i

Clinical significance

Improper functioning is associated with carbamoyl phosphate synthetase I deficiency.

External links

MeSH Carbamoyl-Phosphate+Synthase+(Ammonia)Template:Ligase-stub

v • d • e Metabolism: amino acid metabolism - urea cycle enzymes
Main cycle	mitochondrial matrix: Carbamoyl phosphate synthetase I - Ornithine transcarbamylase cytosol: Argininosuccinate synthetase - Argininosuccinate lyase - Arginase
Regulatory/transport	N-Acetylglutamate synthase - Ornithine translocase
see also disorders

v • d • e Enzymes: ligases (EC 6)
6.1 - Carbon-Oxygen	Aminoacyl tRNA synthetase
6.2 - Carbon-Sulfur	Succinyl coenzyme A synthetase - Acetyl Co-A synthetase - Long fatty acyl CoA synthetase
6.3 - Carbon-Nitrogen	Glutamine synthetase - Ubiquitin ligase (Von Hippel-Lindau tumor suppressor, UBE3A, Mdm2, Anaphase-promoting complex) - Glutathione synthetase - CTP synthase - Adenylosuccinate synthase - Argininosuccinate synthetase - Holocarboxylase synthetase - GMP synthase - Asparagine synthetase - Carbamoyl phosphate synthase (I , II )
6.4 - Carbon-Carbon	Carbon-carbon ligases
6.5 - Phosphoric Ester	DNA ligase

v • d • e Mitochondrial enzymes and transporters
Outer membrane	Long fatty acyl CoA synthetase - Kynureninase - Monoamine oxidase
Intermembrane space	Adenylate kinase - Creatine kinase
Inner membrane	Coenzyme Q - cytochrome c reductase -Cytochrome c - Dihydroorotate dehydrogenase -Glutamate aspartate transporter - Glycerol-3-phosphate dehydrogenase - NADH dehydrogenase - Succinate dehydrogenase
Matrix	Aconitase - Aspartate transaminase - ALDH2 - Carbamoyl phosphate synthase - Citrate synthase - Fumarase - Glutamate dehydrogenase - Isocitrate dehydrogenase - Malate dehydrogenase - Ornithine transcarbamoylase - Oxoglutarate dehydrogenase - Pyruvate dehydrogenase complex - Succinyl coenzyme A synthetase
Mitochondrial DNA	Complex I (7 units) - Complex III (1 unit) - Complex IV (3 units) - ATP synthase (2 units)