Hyperprolinemia type II: identification of the glycine conjugate of pyrrole-2-carboxylic acid in urine.
The urinary organic acids were studied in two cases of hyperprolinemia Type II, using various combinations of chromatographic, electrophoretic and mass spectrometric techniques. In both cases N-(pyrrole-2-carboxylic acid) was idenfitied as a major urinary metabolite. While there was evidence for an additional conjugate of this pyrrolic acid, no free pyrrole-carboxylate could be detected in the urine from either case.
D002264:Carboxylic Acids / Q000652:urine; D002849:Chromatography, Gas; D002855:Chromatography, Thin Layer; D004151:Dipeptides / Q000652:urine*; D004590:Electrophoresis, Paper; D005998:Glycine / Q000652:urine*; D006801:Humans; D008722:Methods; D000587:Oxidoreductases Acting on CH-NH Group Donors / Q000172:deficiency*; D011758:Pyrroles / Q000652:urine*; D011762:Pyrroline Carboxylate Reductases / Q000172:deficiency*
D002264:Carboxylic Acids; D004151:Dipeptides; D011758:Pyrroles; D000587:Oxidoreductases Acting on CH-NH Group Donors; D011762:Pyrroline Carboxylate Reductases; D005998:Glycine