N-Carbamyl-L-Glutamic Acid

N-Carbamyl-L-glutamic acid is an activator of carbamoyl phosphate synthetase 1 (CPS1), the first enzyme in the urea cycle.¹ It is a structural analog of N-acetyl-glutamate, an endogenous CPS1 activator. N-Carbamyl-L-glutamic acid (500 mg/kg) reduces 2-ketoisocaproate-induced hyperammonemia in a mouse model of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.² It inhibits cell proliferation of a variety of cancer cell lines with IC₅₀ values ranging from 5 to 7.5 nM.³ It also inhibits tumor growth by 80 and 82% in orthotopic mouse models of pancreatic and triple-negative breast cancer, respectively, when administered at a dose of 120 mg/kg per day for 10 days. Formulations containing N-carbamyl-L-glutamic acid have been used in the treatment of primary N-acetyl-glutamate synthase deficiency.

WARNING This product is not for human or veterinary use.

1. Häberle, J. Carglumic acid for the treatment of N-acetylglutamate synthase deficiency and acute hyperammonemia. Exp. Rev. Endocrinol. Metab. 7(3), 263-271 (2012).

2. Gauthier, N., Wu, J.W., Wang, S.P., et al. A liver-specific defect of Acyl-CoA degradation produces hyperammonemia, hypoglycemia and a distinct hepatic Acyl-CoA pattern. PLoS One 8(7), e60581 (2013).

3. Chen, C.-T., Chen, Y.-C., Yamaguchi, H., et al. Carglumic acid promotes apoptosis and suppresses cancer cell proliferation in vitro and in vivo. Am. J. Cancer Res. 5(12), 3560-3569 (2015).