Description: Carglumic Acid (Ureidoglutaric acid) is an orphan drug used to treat hyperammonemia in patients with N-acetyl glutamate synthase absence. This rare hereditary disease results in elevated blood levels of ammonia, which can eventually cross the blood-brain barrier and lead to neurologic problems, coma,...

N-Carbamyl-L-glutamic acid is an activator of carbamoyl phosphate synthetase 1 (CPS1), the first enzyme in the urea cycle. It is a structural analog of N-acetyl-glutamate, an endogenous CPS1 activator. N-Carbamyl-L-glutamic acid (500 mg/kg) reduces 2-ketoisocaproate-induced hyperammonemia in a mouse model of...