Structure of the three major fucosyl-glycoasparagines accumulating in the urine of a patient with fucosidosis.

Abstract

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.

• Authors: G Strecker, B Fournet, J Montreuil
• Journal: Biochimie (Biochimie) Vol. 60 Issue 8 Pg. 725-34 ( 1978) ISSN: 0300-9084 [Print] France
• PMID: 728478 (Publication Type: Journal Article)

Chemical References

• Glycopeptides
• Oligosaccharides
• Fucose

Topics

• Carbohydrate Metabolism, Inborn Errors (urine)
• Chemical Phenomena
• Chemistry
• Chromatography, Paper (methods)
• Fucose (metabolism)
• Glycopeptides (urine)
• Humans
• Magnetic Resonance Spectroscopy (methods)
• Mass Spectrometry
• Oligosaccharides (urine)