Carglumic acid

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Scientific Name: Carglumic acid
Brand Name: Carbaglu
Company Owner: Not Available
Mechanism Of Action Carglumic acid is a synthetic structural analogue of N-acetylglutamate (NAG) , which is an essential allosteric activator of the liver enzyme carbamoyl phosphate synthetase 1 (CPS1) . CPS1 is found in the mitochondria and is the first enzyme of the urea cycle, which converts ammonia into urea. Carglumic acid acts as a replacement for NAG in NAGS deficiency patients by activating CPS1 but it does not help to regulate the urea cycle.
Description of the Drug: Carglumic acid is an analog of N-acetylglutamate (NAG) used for the treatment of acute and chronic hyperammonemia in patients with N-acetylglutamate synthase (NAGS) deficiency.
Protein Data Bank: Not Available
Source: DrugBank Online – Retrieved 2023-01-23 from