Isovaleroylglycine is a compound found in the urine of patients with isovaleric acidemia, a disorder created by defective leucine metabolism due to a deficiency in the isovaleryl coenzyme A dehydrogenase.1 It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid β-oxidation defects.
1.Tanaka, K., and Isselbacher, K.J.The isolation and identification of N-isovalerylglycine from urine of patients with isovaleric acidemiaJ. Biol. Chem.242(12)2966-2972(1967)