Description: N-Isovaleroylglycine (Isovaleroylglycine) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases, the measurement of these metabolites in body fluids can be...

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...