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Carglumic acid

Brand names: CARBAGLU


Carglumic acid is a carbamoyl phosphate synthetase 1 (CPS 1) allosteric modulator. 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 N-acetylglutamate (NAG) in N-acetylglutamate synthase (NAGS) deficiency patients by activating CPS1 but it does not help to regulate the urea cycle. Carglumic acid indicated as adjunctive therapy for the treatment of acute hyperammonemia due to the deficiency of the hepatic enzyme NAGS.

ATC Classification

Carglumic acid
A Alimentary tract and metabolism → A16 Other alimentary tract and metabolism products → A16A Other alimentary tract and metabolism products → A16AA Amino acids and derivatives

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