Plays a role in the regulation of ureagenesis by producing the essential cofactor N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPS1) activity. acetyl-CoA + L-glutamate = CoA + H(+) + N-acetyl-L-glutamate Increased by L-arginine. Amino-acid biosynthesis; L-arginine biosynthesis; N(2)-acetyl-L-ornithine from L-glutamate: step 1/4. Homodimer. Homotetramer. Highly expressed in the adult liver, kidney and small intestine. Weakly expressed in the fetal liver, lung, pancreas, placenta, heart and brain tissue. The amino-acid kinase (AAK) domain mediates binding of the allosteric activator L-arginine. Probably processed by mitochondrial processing peptidase (MPP). The long form has not yet been isolated (By similarity). The disease is caused by variants affecting the gene represented in this entry. Belongs to the acetyltransferase family. N-acetylglutamate synthase entry