title: Isolation and structural characterization of twenty-one sialyloligosaccharides from galactosialidosis urine. An intact N,N'-diacetylchitobiose unit at the reducing end of a diantennary structure.
abstract: Galactosialidosis urine was fractionated by gel-permeation chromatography on Bio-Gel P-6. The obtained sialic acid-containing carbohydrate fractions were purified by reversed-phase chromatography and separated according to charge by medium-pressure anion-exchange chromatography on Mono Q. The Mono Q fractions, being mixtures of sialyloligosaccharides differing mainly in sialic acid-linkage type (alpha 2-3/alpha 2-6), were subfractionated by high-performance liquid chromatography on Lichrosorb-NH2. The purified compounds were analysed by 500-MHz 1H-NMR spectroscopy. Twenty-one fully and partially sialylated N-acetyllactosamine-type compounds include mono-, di-, tri- and tetra-antennary structures. All structures have the sequence Man beta 1-4Glc-NAc at the reducing terminus in common, except one diantennary structure bearing an intact N,N'-diacetylchitobiose unit at the reducing end, which is a new feature in human glycoproteinosis urine.
authors.affiliation: Department of Bio-Organic Chemistry, Utrecht University.
authors.forename: J
authors.initials: J
authors.lastname: van Pelt
authors.forename: K
authors.initials: K
authors.lastname: Hård
authors.forename: J P
authors.initials: JP
authors.lastname: Kamerling
authors.forename: J F
authors.initials: JF
authors.lastname: Vliegenthart
authors.forename: A J
authors.initials: AJ
authors.lastname: Reuser
authors.forename: H
authors.initials: H
authors.lastname: Galjaard
mesh_terms: D002850:Chromatography, Gel; D002851:Chromatography, High Pressure Liquid; D004187:Disaccharides; D005936:Glucans / Q000032:analysis / Q000652:urine*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D009439:Neuraminidase / Q000172:deficiency*; D009844:Oligosaccharides / Q000032:analysis / Q000652:urine*
chemical_list: D004187:Disaccharides; D005936:Glucans; D009844:Oligosaccharides; C016735:sialooligosaccharides; C010913:N,N-diacetylchitobiose; D009439:Neuraminidase