Chemical formula: C₆H₁₀N₂O₅ Molecular mass: 190.154 g/mol PubChem compound: 121396
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.
This medicinal substance has been classified in the anatomical therapeutic chemical (ATC) classification according to its main therapeutic use as follows:
| ATC code | Group title | Classification |
|---|---|---|
| A16AA05 | 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 |
Competent medicine agencies globally have authorized commercialization of this active ingredient according to these medication package inserts (MPIs):
| Title | Information Source | Document Type | |
|---|---|---|---|
| CARBAGLU Dispersible tablet | European Medicines Agency (EU) | MPI, EU: SmPC |
Carglumic acid is an active ingredient of these brands:
United States (US)Brazil (BR)Canada (CA)Israel (IL)Japan (JP)New Zealand (NZ)Romania (RO)Note the following: The list of brand names is continuously updated, and thus does not include the total of products circulating worldwide. |
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