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Bruggink, C.; Poorthuis, B.J.H.M.; Piraud, M.; Froissart, R.; Deelder, A.M.; Wuhrer, M. (2010)

Glycan profiling of urine, amniotic fluid and ascitic fluid from galactosialidosis patients reveals novel oligosaccharides with reducing end hexose and aldohexonic acid residues

Article / Letter to editor
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-beta-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:...Show moreUrine, 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-beta-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.Show less
catabolism clinical glycomics HPAEC-PAD mass spectrometry metabolic disorder anion-exchange chromatography pulsed amperometric detection tandem mass-spectrometry beta-galactosidase human-milk structural characterization neuraminidase deficiency sialidosis fibroblasts escherichia-coli keratan sulfate
All authors
Bruggink, C.; Poorthuis, B.J.H.M.; Piraud, M.; Froissart, R.; Deelder, A.M.; Wuhrer, M.
Date
2010-07-31
Journal
FEBS Journal
Volume
277
Issue
14
Pages
2970 - 2986
DOI
doi:10.1111/j.1742-4658.2010.07707.x