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  • Title: Sialogluciduria in lysosomal diseases: quantitative and qualitative analysis of urinary low molecular sialoglucides from patients with mucopolysaccharidosis and with mucolipidosis.
    Author: Koseki M, Wu JY, Tsurumi K, Nagai Y.
    Journal: Tohoku J Exp Med; 1978 Apr; 124(4):351-60. PubMed ID: 96548.
    Abstract:
    Low molecular sialoglucides were isolated from the urines of normal human male and two patients with lysosomal disease (mucopolysaccharidosis type II and a new type of mucolipidosis) by charcoal adsorption method. Urinary sialoglucides were fractionated into two fractions (SG-1 and SG-2) by Sephadex G-25 gel filtration and considerable increase in excretion of SG-1 was observed in the patients with lysosomal diseases: two- to three-fold increase in mucopolysaccharidosis type II and seven- to eight-fold increase in mucolipidosis. SG-1 was further fractionated into 18 to 19 fractions by Sephadex G-50 gel filtration and ion exchange chromatography. Comparison of the amounts and the chemical compositions of these fractions suggested that the increase in SG-1 was dependent upon the increase in excretion of low molecular sialoglucides rich in mannose and N-acetylglucosamine.
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