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Carglumic acid

Carglumic acid
Systematic (IUPAC) name
N-Carbamoyl-L-glutamic Acid
CAS number 1188-38-1
ATC code A16AA05
PubChem  ?
Chemical data
Formula C6H10N2NaO5 
Mol. mass 190.2 g/mol
Pharmacokinetic data
Bioavailability 30%
Protein binding Undetermined
Metabolism Partial
Half life 4.3 to 9.5 hours
Excretion Fecal (60%) and renal (9%, unchanged)
Therapeutic considerations
Licence data


Pregnancy cat.


Legal status
Routes Oral

Carglumic acid is an orphan drug, marketed by Orphan Europe under the trade name Carbaglu. Carglumic acid is used for the treatment of hyperammonaemia in patients with N-acetylglutamate synthase deficiency. The initial daily dose ranges from 100 to 250 mg/kg, adjusted thereafter to maintain normal plasma levels of ammonia.


  • Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M (2004). "Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate". J Pediatr 145 (4): 552–4. PMID 15480384.
  • Elpeleg O, Shaag A, Ben-Shalom E, Schmid T, Bachmann C (2002). "N-acetylglutamate synthase deficiency and the treatment of hyperammonemic encephalopathy". Ann Neurol 52 (6): 845–9. PMID 12447942.
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Carglumic_acid". A list of authors is available in Wikipedia.
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