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  • Title: Accumulation of mannosyl-beta(1----4)-N-acetylglucosamine in fibroblasts and leukocytes of patients with a deficiency of beta-mannosidase.
    Author: van Pelt J, Hokke CH, Dorland L, Duran M, Kamerling JP, Vliegenthart JF.
    Journal: Clin Chim Acta; 1990 Jan 31; 187(1):55-60. PubMed ID: 2317928.
    Abstract:
    Cultured fibroblasts from two brothers with beta-mannosidosis were shown to accumulate the disaccharide mannosyl-beta(1----4)-N-acetylglucosamine in amounts of 52 and 68 nmol/mg protein. Structural identification of the Man beta(1----4)GlcNAc, isolated by HPLC, was performed by 500-MHz 1H-NMR spectroscopy and sugar composition analysis. Control fibroblasts did not contain a detectable amount of Man beta(1----4)GlcNAc. The disaccharide was also present in leukocytes from these patients, but not in controls, as could be demonstrated by thin-layer chromatography and sugar composition analysis. However, the amounts in leukocytes (5 and 6 nmol/mg protein) were much smaller than those in fibroblasts.
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