Carbaglu 是一种 N-乙酰谷氨酸 (NAG) 的功能类似物和氨甲酰磷酸合成酶 1(CPS1)的活化剂，可用于与 N-乙酰谷氨酸合成酶 (NAGS) 缺陷相关的急性和慢性高氨血症的研究。

Carglumic acid is an orphan drug used to treat hyperammonemia in patients with N-acetyl glutamate synthase absence. This rare hereditary disease results in elevated blood levels of ammonia, which can eventually cross the blood-brain barrier and lead to neurologic problems, coma, cerebral edema, and death. Carglumic acid was approved by the U.S. FDA on 18 March 2010.

1188-38-1

C6H10N2O5

190.155

Carbamino-L-glutamic acid;Carbaglu;Ureidoglutaric acid;N-Carbamylglutamate;Carbamylglutamic acid;氨甲酰谷氨酸;N-Carbamyl-L-glutamic acid;Carglumic Acid

DMSO:199.8 mM
Powder: -20°C for 3 years
In solvent: -80°C for 2 years