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

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-b-N-acetylglucosaminidase-cleaved products of complex-type sialylated N-glycans, O-sulfated oligosaccharide moieties were detected. | Glycan profiling of urine amniotic fluid and ascitic fluid from galactosialidosis patients reveals novel oligosaccharides with reducing end hexose and aldohexonic acid residues Cees Bruggink1 2 Ben J. H. M. Poorthuis3 Monique Piraud4 Roseline Froissart4 Andre M. Deelder1 and Manfred Wuhrer1 1 Biomolecular Mass Spectrometry Unit Department of Parasitology Leiden University MedicalCenter Leiden The Netherlands 2 Dionex Benelux BV Amsterdam The Netherlands 3 Department of MedicalBiochemistry Academic MedicalCenter Amsterdam The Netherlands 4 Laboratoire des Maladies Héréditaires du Metabolisme et Depistage Neonatal Centre de Biologie Est Hospices Civils de Lyon Bron France Keywords catabolism clinicalglycomics HPAEC-PAD mass spectrometry metabolic disorder Correspondence C. Bruggink Biomolecular Mass Spectrometry Unit Department of Parasitology Leiden University Medical Center PO Box 9600 2300 RC Leiden The Netherlands Fax 31 71 5266907 Tel 31 71 5266079 E-mail c.bruggink@lumc.nl Website http www.lumc.nl con 1040 81028091348221 811071049172556 902270938532556 811120200332556 Received 4 March 2010 revised 16 April 2010 accepted 11 May 2010 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-b-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-ace-tylhexosamine-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 Ci-oxidized aldohexonic acidcontaining versions of most of these .