Chromatographic determination and mass spectrometric identification of gamma-glutamylphenylalanine, a urinary constituent in phenylketonuria.
The occurrence of gamma-glutamylphenylalanine in the urine of patients with phenylketonuria could be demonstrated using chromatographic techniques and mass spectrometry. Concentrations ranged up to 35 mg/l. Only a weak correlation between the urinary excretion of this compound and phenylalanine was seen. The ages of the patients investigated ranged from 2 weeks to 18 years. The origin of the dipeptide is discussed.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002849:Chromatography, Gas; D002855:Chromatography, Thin Layer; D004151:Dipeptides / Q000652:urine*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D013058:Mass Spectrometry; D010649:Phenylalanine / Q000097:blood / Q000652:urine; D010661:Phenylketonurias / Q000652:urine*
D004151:Dipeptides; D010649:Phenylalanine; C020621:gamma-glutamylphenylalanine