| carbamoyl-phosphate synthase (ammonia) |
[EC 6.3.4.16] an enzyme of the ligase class that catalyzes the synthesis of carbamoyl phosphate from ammonia and carbon dioxide, the first committed step in the urea cycle (see illustration at urea cycle, under cycle). The reaction occurs predominantly in liver mitochondria, requires N-acetylglutamate as a cofactor, and hydrolyzes two molecules of ATP. Decreased enzyme activity, an autosomal recessive trait, causes carbamoyl phosphate synthetase deficiency.
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