Automated description from the Alliance of Genome Resources (Release 7.0.0)
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).