N-Carbamyl-L-glutamic acid, a deacylase-resistant analogue of N-acetylglutamate, is a carbamyl phosphate synthetase 1 (CPS1) activator. N-Carbamyl-L-glutamic acid is used to treat primary N-acetyl-L-glutamate synthase deficiency and hyperammonemia associated with organic acidemias such as methylmalonic aciduria, propionic aciduria, and isovaleric aciduria.

Keywords: N-Carbamyl-L-glutamic acid | Supplier | CPS1 activator | Carglumic acid | Carbamylglutamate | CAS [1188-38-1] | [21133-43-7] | [2226892-59-5] | Ammonia | Urea | CPS | Activator | Enzymes | CPS1 deficiency | CPS1D