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Sialyl-alpha 2-6-mannosyl-beta 1-4-N-acetylglucosamine, a novel compound occurring in urine of patients with beta-mannosidosis.
van Pelt, J; Dorland, L; Duran, M; Hokke, C H; Kamerling, J P; Vliegenthart, J F.
Affiliation
  • van Pelt J; University Children's Hospital Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.
J Biol Chem ; 265(32): 19685-9, 1990 Nov 15.
Article in En | MEDLINE | ID: mdl-2246252
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.
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Collection: 01-internacional Database: MEDLINE Main subject: Trisaccharides / Mannosidases Limits: Humans / Male Language: En Journal: J Biol Chem Year: 1990 Document type: Article Affiliation country: Netherlands
Search on Google
Collection: 01-internacional Database: MEDLINE Main subject: Trisaccharides / Mannosidases Limits: Humans / Male Language: En Journal: J Biol Chem Year: 1990 Document type: Article Affiliation country: Netherlands