N-Acetylglutamate synthase

N-Acetylglutamate synthase
N-Acetylglutamate synthase
Identifiers
Symbol NAGS
Entrez 162417
HUGO 17996
OMIM 608300
RefSeq NM_153006
UniProt Q8N159
Other data
EC number 2.3.1.1
Locus Chr. 17 q21.31

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

This enzyme is important in bacteria and plants for the synthesis of arginine, an amino acid in the pathway.

This enzyme is also 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.


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