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ATC Group: A16AA05 Carglumic acid

Anatomical Therapeutic Chemical Classification System


Carglumic acid

Hierarchical Position

Active Ingredients

Chemical substance

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.