1838. Carglumic acid

Nomenclature

CAS number: 1188-38-1
N-(Aminocarbonyl)-l-glutamic acid; carbamylglutamic acid; N-carbamoyl-l-glutamic acid; l-uramidoglutaric acid; ureidoglutaric acid; Carbaglu (Orphan Europe).
C6H10N2O5; mol wt 190.15.
C 37.90%, H 5.30%, N 14.73%, O 42.07%.

Description and 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. Inherit. Metab. Dis. 19, 220 (1996); of N-acetylglutamate synthetase (NAGS) deficiency: B. Plecko et al., Eur. J. Pediatr. 157, 996 (1998).

Chemical structure

Properties

mp 174°.

Therapeutic Category

In treatment of inherited urea cycle disorders.