Carbamoyl phosphate synthase
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carbamoyl-phosphate synthetase 1, mitochondrial
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| Identifiers | |
| Symbol | CPS1 |
| HUGO | 2323 |
| Entrez | 1373 |
| OMIM | 608307 |
| RefSeq | NM_001875 |
| UniProt | P31327 |
| Other data | |
| EC number | 6.3.4.16 |
| Locus | Chr. 2 p |
Carbamoyl phosphate synthase (EC 6.3.5.5, formerly known as EC 2.7.2.9) is the enzyme responsible for catalyzing the reactions which produce Carbamoyl phosphate.
It takes part in these reactions:
- In the urea cycle:
2 ATP + HCO3- + NH3 → carbamoyl phosphate + 2 ADP + Pi
- In pyrimidine biosynthesis:
Gln + CO2 + 2 ATP + H2O → carbamoyl phosphate + Glu + 2 ADP + Pi
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Carbamoyl phosphate synthase - Ornithine transcarbamylase - Argininosuccinate synthetase- Argininosuccinate lyase - Arginase
