16284-60-9

White to Off-White Solid

N-Isovalerylglycine is a useful diagnostic tool in the diagnosis of many organic acidemias and mitochondrial fatty acid β-oxidation defects. N-Isovalerylglycine is found in the urine of patients with isovaleric acidemia, an inborn error of leucine metabolism.

ChEBI: An N-acylglycine in which the acyl group is specified as isovaleryl.

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. It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid β-oxidation defects.

[1] K. TANAKA K I. The isolation and identification of N-isovalerylglycine from urine of patients with isovaleric acidemia.[J]. The Journal of Biological Chemistry, 1967, 55 1: 2966-2972. DOI: 10.1016/s0021-9258(18)99599-2