title: 2-Acetamidoglucal, a new metabolite isolated from the urine of a patient with sialuria.
abstract: A new metabolite, namely 2-acetamidoglucal, has been found in the urine of a patient with sialuria in addition to the metabolites N-acetylneuraminic acid, N-acetylmannosamine, N-acetylglucosamine and 2-deoxy-2,3-dehydro-N-acetylneuraminic acid reported earlier. the structure has been identified by mass spectrometry and 360 MHz proton nuclear magnetic resonance spectroscopy and verified by synthesis. All accumulated compounds fit into the metabolic pathway for the biosynthesis of CMP-N-acetylneuraminic acid. Sialuria is discussed in terms of a failure of regulation of UDP-N-acetylglucosamine 2-epimerase.
mesh_terms: D000117:Acetylglucosamine / Q000652:urine; D002239:Carbohydrate Metabolism, Inborn Errors / Q000209:etiology / Q000378:metabolism / Q000652:urine*; D003569:Cytidine Monophosphate N-Acetylneuraminic Acid / Q000378:metabolism; D006595:Hexosamines / Q000032:analysis / Q000652:urine*; D006801:Humans; D008297:Male; D009005:Monosaccharides / Q000652:urine*; D012794:Sialic Acids / Q000652:urine*
chemical_list: D006595:Hexosamines; D009005:Monosaccharides; D012794:Sialic Acids; C019231:2-acetamidoglucal; D003569:Cytidine Monophosphate N-Acetylneuraminic Acid; D000117:Acetylglucosamine