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Carglumic acid


Carglumic acid
Systematic (IUPAC) name
N-Carbamoyl-L-glutamic Acid
Identifiers
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	EU
Pregnancy cat.	unknown
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.

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References

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.

v • d • e Other alimentary tract and metabolism products (A16)
Amino acids and derivatives	Levocarnitine - Ademetionine - Levoglutamide - Mercaptamine - Carglumic acid - Betaine
Enzymes	Alglucerase - Imiglucerase - Agalsidase alfa - Agalsidase beta - Laronidase - Sacrosidase - Alglucosidase alfa - Galsulfase - Idursulfase
Various alimentary tract and metabolism products	Tioctic acid - Anethole trithione - Sodium phenylbutyrate - Nitisinone - Zinc acetate - Miglustat