| carbamoyl-phosphate synthetase 1, mitochondrial | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | CPS1 | ||||||
| NCBI gene | 1373 | ||||||
| HGNC | 2323 | ||||||
| OMIM | 608307 | ||||||
| RefSeq | NM_001875 | ||||||
| UniProt | P31327 | ||||||
| Other data | |||||||
| EC number | 6.3.4.16 | ||||||
| Locus | Chr. 2 p | ||||||
| |||||||
Carbamoyl phosphate synthetase I (CPS I) is a ligase enzyme located in the mitochondria involved in the production of urea. Carbamoyl phosphate synthetase I (CPS1 or CPSI) transfers an ammonia molecule to a molecule of bicarbonate that has been phosphorylated by a molecule of ATP. The resulting carbamate is then phosphorylated with another molecule of ATP. The resulting molecule of carbamoyl phosphate leaves the enzyme.