Isolation and identification of 2-oxo-5-guanidinovaleric acid in urine of patients with hyperargininaemia by chromatography and gas chromatography/mass spectrometry.
An unknown guanidino-positive peak has been identified in urines of three sisters affected with hyperargininaemia. Identification was made on the basis of its similarity with the liquid and thin-layer chromatographic characteristics of enzymatically synthesized 2-oxo-5-guanidinovaleric acid. Identification was also made by combined gas chromatography-mass spectrometry of the unknown compound peak. The synthesis of enzymatically formed 2-oxo-5-guanidinovaleric acid was controlled by nuclear magnetic resonance and combined gas chromatography-mass spectrometry.
D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine*; D001120:Arginine / Q000097:blood*; D055598:Chemical Phenomena; D002621:Chemistry; D002853:Chromatography, Liquid; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D006146:Guanidines / Q000652:urine*; D006801:Humans; D007651:Keto Acids / Q000652:urine*; D009682:Magnetic Resonance Spectroscopy
D006146:Guanidines; D007651:Keto Acids; C022249:alpha-keto-delta-guanidinovaleric acid; D001120:Arginine