title: Sialyl-alpha 2-6-mannosyl-beta 1-4-N-acetylglucosamine, a novel compound occurring in urine of patients with beta-mannosidosis.
abstract: Human beta-mannosidosis urine was fractionated by gel permeation chromatography on Bio-Gel P-2 and by high performance liquid chromatography on Partisil 10 SAX. Besides the disaccharide Man beta 1-4GlcNAc as the major component, a sialic acid-containing compound was detected in an amount of 10% compared to that of Man beta 1-4GlcNAc. Structural characterization of the oligosaccharide and of its reduced analogue by sugar composition analysis, methylation analysis, gas-liquid chromatography-mass spectrometry, and 500-MHz 1H NMR spectroscopy gave conclusive evidence for a novel urinary constituent: NeuAc alpha 2-6Man beta 1-4GlcNAc. This linear trisaccharide can be considered as the result of an alpha 2-6-sialylation of the major accumulating compound, Man beta 1-4GlcNAc. The hitherto unknown linkage between sialic acid and mannose was shown to be susceptible to sialidase digestion.
authors: University Children's Hospital Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.
authors: J
authors: J
authors: van Pelt
authors: L
authors: L
authors: Dorland
authors: M
authors: M
authors: Duran
authors: C H
authors: CH
authors: Hokke
authors: J P
authors: JP
authors: Kamerling
authors: J F
authors: JF
authors: Vliegenthart
mesh_terms: D002240:Carbohydrate Sequence; D002241:Carbohydrates / Q000032:analysis; D002850:Chromatography, Gel; D002851:Chromatography, High Pressure Liquid; D002855:Chromatography, Thin Layer; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008358:Mannose / Q000032:analysis / Q000378:metabolism; D008361:Mannosidases / Q000172:deficiency*; D008745:Methylation; D008969:Molecular Sequence Data; D019158:N-Acetylneuraminic Acid; D012794:Sialic Acids / Q000032:analysis / Q000378:metabolism; D014312:Trisaccharides / Q000737:chemistry / Q000652:urine*; D044902:beta-Mannosidase
chemical_list: D002241:Carbohydrates; D012794:Sialic Acids; D014312:Trisaccharides; C066483:sialyl-alpha2-6-mannosyl-beta1-4-N-acetylglucosamine; D008361:Mannosidases; D044902:beta-Mannosidase; D019158:N-Acetylneuraminic Acid; D008358:Mannose