CAS 56613-60-6: N-(3-phenylpropanoyl)glycine

N-(3-Phenylpropionyl)glycine

N-(3-Phenylpropionyl)glycine, an acyl glycine, indicates medium-chain acyl-CoA dehydrogenase deficiency when detected in urine.

N-(3-Phenylpropionyl)glycine is a metabolite of the amino acid glycine that is produced by plants. It has been used in diagnostic tests to measure the activity of enzymes involved in fatty acid metabolism and as a marker for metabolic disorders. N-(3-Phenylpropionyl)glycine can be detected in urine samples and has been used to diagnose different types of orotic aciduria, including infantile orotic aciduria. This metabolite inhibits the activity of the enzyme hexanoylglycerol O-acyltransferase, which catalyzes the conversion of hexanoylglycine to p-hydroxybenzoic acid. Hexanoylglycine is an intermediate product of human liver metabolism and it can be found in high concentrations in body tissues such as heart tissue.

Applications N-(3-Phenylpropionyl)glycine-3C2,15N is a labelled analogue of N-(3-Phenylpropionyl)glycine (P336210), which is a useful diagnostic tool in the diagnosis of many organic acidemias and mitochondrial fatty acid β-oxidation defects. N-(3-Phenylpropionyl)glycine is present in the urine of medium-chain acyl-CoA dehydrogenase deficient (MCAD) patients who orally ingested 3-phenylpropionic acid. References Duran, M. et al.: Prog. Clin. Biol. Res., 321, 419 (1988); Bonafe, L. et al.: Mol. Gen. Metab., 69, 311 (2000); Ombrone, D. et al.: Anal. Biochem., 417, 122 (2011);