Carglumic acid

Title: Carglumic acid
CAS Registry Number: 1188-38-1
CAS Name: N-(Aminocarbonyl)-L-glutamic acid
Additional Names: carbamylglutamic acid; N-carbamoyl-L-glutamic acid; l-uramidoglutaric acid; ureidoglutaric acid
Trademarks: Carbaglu (Orphan Europe)
Molecular Formula: C6H10N2O5
Molecular Weight: 190.15
Percent Composition: C 37.90%, H 5.30%, N 14.73%, O 42.07%
Literature References: Metabolically stable analog of N-acetylglutamate, a physiological activator of the first enzyme of the urea cycle, carbamylphosphate synthetase (CAPS). Prepn: H. McIlwain, Biochem. J. 33, 1942 (1939). Effect on blood urea and ammonia levels and potential clinical application: J.-E. O'Connor et al., Eur. J. Pediatr. 143, 196 (1985). Evaluation in treatment of CAPS deficiency: G. Kuchler et al., J. Inher. Metab. Dis. 19, 220 (1996); of N-acetylglutamate synthetase (NAGS) deficiency: B. Plecko et al., Eur. J. Pediatr. 157, 996 (1998).
Properties: mp 174°.
Melting point: mp 174°
Therap-Cat: In treatment of inherited urea cycle disorders.
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Carglumic acid
Carglumic acid.svg
Systematic (IUPAC) name
(2S)-2-(carbamoylamino)pentanedioic acid
Clinical data
AHFS/ Consumer Drug Information
Licence data EMA:Link
Pregnancy cat. unknown
Legal status  ?
Routes Oral
Pharmacokinetic data
Bioavailability 30%
Protein binding Undetermined
Metabolism Partial
Half-life 4.3 to 9.5 hours
Excretion Fecal (60%) and renal (9%, unchanged)
CAS number 1188-38-1 N
ATC code A16AA05
PubChem CID 121396
DrugBank DB06775
ChemSpider 108351 YesY
KEGG D07130 YesY
Synonyms (S)-2-ureidopentanedioic acid
Chemical data
Formula C6H10N2O5 
Mol. mass 190.2 g/mol
 N (what is this?)  (verify)

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.[1][2] The initial daily dose ranges from 100 to 250 mg/kg, adjusted thereafter to maintain normal plasma levels of ammonia.

The US FDA approved it for treatment of hyperammonaemia on March 18, 2010. Orphan Drug exclusivity expires on March 18,2017.[3]