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Glycan profiling of urine, amniotic fluid and ascitic fluid from galactosialidosis patients reveals novel oligosaccharides with reducing end hexose and aldohexonic acid residues
Author(s) -
Bruggink Cees,
Poorthuis Ben J. H. M.,
Piraud Monique,
Froissart Roseline,
Deelder André M.,
Wuhrer Manfred
Publication year - 2010
Publication title -
the febs journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.981
H-Index - 204
eISSN - 1742-4658
pISSN - 1742-464X
DOI - 10.1111/j.1742-4658.2010.07707.x
Subject(s) - chemistry , glycan , oligosaccharide , biochemistry , hexose , sialic acid , enzyme , glycoprotein
Urine, amniotic fluid and ascitic fluid samples of galactosialidosis patients were analyzed and structurally characterized for free oligosaccharides using capillary high‐performance anion‐exchange chromatography with pulsed amperometric detection and online mass spectrometry. In addition to the expected endo‐β‐ N‐ acetylglucosaminidase‐cleaved products of complex‐type sialylated N ‐glycans, O ‐sulfated oligosaccharide moieties were detected. Moreover, novel carbohydrate moieties with reducing‐end hexose residues were detected. On the basis of structural features such as a hexose– N ‐acetylhexosamine–hexose–hexose consensus sequence and di‐sialic acid units, these oligosaccharides are thought to represent, at least in part, glycan moieties of glycosphingolipids. In addition, C 1 ‐oxidized, aldohexonic acid‐containing versions of most of these oligosaccharides were observed. These observations suggest an alternative catabolism of glycosphingolipids in galactosialidosis patients: oligosaccharide moieties from glycosphingolipids would be released by a hitherto unknown ceramide glycanase activity. The results show the potential and versatility of the analytical approach for structural characterization of oligosaccharides in various body fluids.