Sialogluciduria in Lysosomal Diseases: Quantitative and Qualitative Analysis of Urinary Low Molecular Sialoglucides from Patients with Mucopolysaccharidosis and with Mucolipidosis

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Abstract
Low molecular sialoglucides were isolated from the urines of normal human male and 2 patients with lysosomal disease (mucopolysaccharidosis type II and a new type of mucolipidosis) by charcoal adsorption method. Urinary sialoglucides were fractionated into 2 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: 2- to 3-fold increase in mucopolysaccharidosis type II and 7- to 8-fold increase in mucolipidosis. SG-1 was further fractionated into 18-19 fractions by Sephadex G-50 gel filtration and ion exchange chromatography. Comparison of the amounts the 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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