Nags - N-acetylglutamate synthase

Enables acetyl-CoA:L-glutamate N-acetyltransferase activity. Acts upstream of or within glutamate metabolic process. Located in mitochondrion. Human ortholog(s) of this gene implicated in N-acetylglutamate synthase deficiency and amino acid metabolic disorder. Orthologous to human NAGS (N-acetylglutamate synthase). [provided by Alliance of Genome Resources, Apr 2022]