N-Acetylglutamate synthase

N-acetylglutamate synthase is an enzyme which catalyses the production of N-acetylglutamate from acetyl-CoA and glutamate.

This enzyme is important in bacteria and plants for the synthesis of arginine, because it is part of this pathway.

Also this enzyme is important for mammals, because it produces the regulator of urea cycle N-acetylglutamate, which activates carbamoyl phosphate synthetase I, which catalyses the initial reactions of urea cycle.

Clinical significance
Mutations in the gene are associated with N-Acetylglutamate synthase deficiency.