Carbamoyl phosphate synthase I

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.