Carbamoyl phosphate synthase I

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carbamoyl-phosphate synthetase 1, mitochondrial
Identifiers
SymbolCPS1
Entrez1373
HUGO2323
OMIM608307
RefSeqNM_001875
UniProtP31327
Other data
EC number6.3.4.16
LocusChr. 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 + Pi

Clinical significance

Improper functioning is associated with carbamoyl phosphate synthetase I deficiency.

External links

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