Glycan profiling of urine, amniotic fluid and ascitic fluid from galactosialidosis patients reveals novel oligosaccharides with reducing end hexose and aldohexonic acid residues.
FEBS J
; 277(14): 2970-86, 2010 Jul.
Article
in En
| MEDLINE
| ID: mdl-20546307
ABSTRACT
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 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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Oligosaccharides
/
Ascitic Fluid
/
Lysosomal Storage Diseases
/
Beta-Galactosidase
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Amniotic Fluid
/
Neuraminidase
Limits:
Humans
/
Infant
/
Newborn
Language:
En
Journal:
FEBS J
Journal subject:
BIOQUIMICA
Year:
2010
Document type:
Article
Affiliation country:
Netherlands