An activator of carbamoyl phosphate synthetase 1; reduces 2-ketoisocaproate-induced hyperammonemia in a mouse model of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency when used at a dose of 500 mg/kg; inhibits cancer cell proliferation (IC50s = 5-7.5 nM) and tumor growth (120 mg/kg per day for 10 days) in mouse models of pancreatic and breast cancer