Propionylglycine 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 used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme th at catalyzes the chemical reaction:acyl-CoA + glycine < -- > CoA + N-acylglycine. The level of propionylglycine is elevated in patients with propionic acidemia, which is an inherited deficiency of propionyl-CoA carboxylase.
