N-(3-Phenylpropionyl)glycine detection 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.
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