Carbamoyl-Phosphate Synthase [Ammonia], Mitochondrial

**Semantic type:** Amino Acid, Peptide, or Protein|Enzyme

**Definition:** Carbamoyl-phosphate synthase [ammonia], mitochondrial (1500 aa, ~165 kDa) is encoded by the human CPS1 gene. This protein plays a role in the synthesis of carbamoyl phosphate from ammonia and bicarbonate.

**Synonyms:** - CPS1 - CPSase I - Carbamoyl-Phosphate Synthase 1 - Carbamoyl-Phosphate Synthase 1, Mitochondrial - Carbamoyl-Phosphate Synthetase I - EC 6.3.4.16