N-Acetyl-L-Glutamate

N-Acetyl-L-Glutamate is an endogenous amino acid derivative that functions as a crucial metabolic intermediate, particularly in bacterial arginine biosynthesis. It is also recognized as an essential allosteric activator of carbamoyl phosphate synthetase 1 (CPS1), the initial and rate-limiting enzyme in the urea cycle, which is critical for the detoxification of ammonia. Deficiency in N-Acetyl-L-Glutamate is associated with N-acetylglutamate synthase deficiency (NAGSD), a urea cycle disorder. Its levels are observed to be altered in various physiological and pathological states, including ulcerative colitis, Parkinson's disease, diabetic retinopathy, hyperuricemia, and in response to immune stress or specific dietary interventions.

Other names

N-acetylglutamateN-acetyl-L-glutamic acid