Sialogluciduria in Lysosomal Diseases: Quantitative and Qualitative Analysis of Urinary Low Molecular Sialoglucides from Patients with Mucopolysaccharidosis and with Mucolipidosis
| Autor: | Yasushi Nagai, Masai Koseki, Koichi Tsurumi, Jin-Yeh Wu |
|---|---|
| Rok vydání: | 1978 |
| Předmět: |
Male
Chromatography Gas Mucolipidosis Chemistry Sialoglycoproteins Urinary system Mucopolysaccharidosis Size-exclusion chromatography Oligosaccharides Mannose General Medicine Mucopolysaccharidoses medicine.disease General Biochemistry Genetics and Molecular Biology Excretion chemistry.chemical_compound Biochemistry Mucolipidoses Sephadex Sialic Acids medicine Humans Amino Acids Mucopolysaccharidosis type II Child |
| Zdroj: | The Tohoku Journal of Experimental Medicine. 124:351-360 |
| ISSN: | 1349-3329 0040-8727 |
| DOI: | 10.1620/tjem.124.351 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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