Structure of the three major fucosyl-glycoasparagines accumulating in the urine of a patient with fucosidosis.
Fifteen fucosyl-oligosaccharides and fucosyl-glycoasparagines have been isolated from the urine of a patient with fucosidosis. The structure of the three most abundant glycoasparagines are as follows: alpha-Fuc-(1 lead to 6)-beta-GlcNAc-Asn; alpha-Man-(1 leads to 6)-beta-Man-(1 leads to 4)-beta-GlcNAc-(1 lead to 4) [alpha-Fuc-(1 leads to 6)]-belta-GlcNAc-Asn; beta-Gal-(1 leads to 4) [alpha-Fuc-(1 lead to 3)] beta-GlcNAc-(1 leads to 2)-alpha-Man-(1 lead to 6)-beta-Man-(1 leads to 4)-beta-GlcNAc-(1 leads to 4) [alpha-Fuc-(1 leads to 6)] beta-GlcNAc-Asn. The structures are related to the class of fucosyl-glycoproteins (e.g.: IgG immunoglobulin, lactotransferrin and alpha 1-acid glycoprotein). The terminal sequence: beta-Gal-(1 leads to 4) [alph-Fuc-(1 leads to 3)] beta-GlcNAc-(1 leads to 2)-a-Man leads to R is novel for carbohydrate moieties in glycoproteins.
D002239:Carbohydrate Metabolism, Inborn Errors / Q000652:urine*; D055598:Chemical Phenomena; D002621:Chemistry; D002854:Chromatography, Paper / Q000379:methods; D005643:Fucose / Q000378:metabolism*; D006020:Glycopeptides / Q000652:urine*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D013058:Mass Spectrometry; D009844:Oligosaccharides / Q000652:urine
D006020:Glycopeptides; D009844:Oligosaccharides; D005643:Fucose