Characterization and 400-MHz 1H-NMR analysis of urinary fucosyl glycoasparagines in fucosidosis.
Fucosyl glycoasparagines accumulating in the urine of a patient with fucosidosis were isolated using reverse-phase HPLC. Structural analysis of 25 glycoasparagines was carried out by combination of methylation and 400-MHz 1H-NMR spectroscopy analyses. The compounds represent different steps in the incomplete catabolism of N-glycosidically linked glycans, as the result of an alpha-L-fucosidase deficiency. All of the glycoasparagines possess a fucose residue alpha-1,6-linked to the GlcNAc 1 residue attached to asparagine. Fucose residues on the peripheral branches were linked either alpha-1,3 to GlcNAc residues (X determinant) or alpha-1,2 to galactose residues (H determinant). The present study allows precise assignments of the NMR parameters for most of the fucosyl linkages occurring in N-glycosidically linked glycans of the N-acetyllactosamine type.
Laboratoire de Chimie Biologique, Université des Sciences et Techniques de Lille Flandres-Artois, Villeneuve d'Ascq, France.
J C
JC
Michalski
J M
JM
Wieruszeski
C
C
Alonso
P
P
Cache
J
J
Montreuil
G
G
Strecker
D002240:Carbohydrate Sequence; D002851:Chromatography, High Pressure Liquid; D005643:Fucose / Q000031:analogs & derivatives / Q000652:urine*; D005645:Fucosidosis / Q000652:urine*; D006020:Glycopeptides / Q000737:chemistry / Q000652:urine*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008745:Methylation; D008969:Molecular Sequence Data
D006020:Glycopeptides; C007872:glycoasparagines; D005643:Fucose