glutamate + acetyl CoA => N-acetyl glutamate + CoA Authored: D'Eustachio, P, 2003-06-24 00:00:00 EC Number: 2.3.1.1 Edited: D'Eustachio, P, 2003-06-24 00:00:00 Mitochondrial N acetylglutamate synthetase (NAGS) catalyzes the reaction of glutamate and acetyl-CoA to form N-acetylglutamate and CoA. NAGS is activated by arginine and the N-acetylglutamate produced in the reaction in turn is required to activate carbamoyl synthetase I. Consistent with this regulatory role in urea synthesis, NAGS mutations in humans are associated with hyperammonemia (Caldovic et al. 2002; Morizono et al. 2004). Pubmed12459178 Pubmed15050968 Reactome Database ID Release 4370542 Reactome, http://www.reactome.org ReactomeREACT_2139