Urinary Oligosaccharides of Fucosidosis

Abstract

Urine of a fucosidosis patient contained a large amount of fucosyl oligosaccharides and fucose-rich glycopeptides. Six major oligosaccharides were purified by a combination of Bio-Gel P-2 and P-4 column chromatographies and paper chromatography. Structural studies by sequential exoglycosidase digestion and by methylation analysis revealed that their structures were as follows: Fucαl→ 6GlcNAc, Fucαl→2Galβ1→4Glc, Galβ1→4(Fucαl→3)GlcNAcβ1→2Manα1→3Manβ1→ 4GlcNAc, Galβ1→4(Fucαl→3)GlcNAcβl→4Manαl→3Manβl→4GlcNAc, Galβl→ 4(Fucαl→3)GlcNAc;β1→2Manαl→6Manβl→4GlcNAc, and Galβ1→4(Fucαl→3)GlcNAc)β1→4Manαl→6Manβ1→4GlcNAc. In addition, the structure of a minor decasaccharide was found to be Galβ1→(Fucαl→)GlcNAcβl→Manαl→[Galβl→(Fucαl→)GlcNAcβ1→Manαl→]Manβ1→4GlcNAc.