尿糖胺聚糖在维尔纳综合征中的分子量依赖性分布

Katsumi Murata
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引用次数: 3

摘要

采用软骨素酶和透明质酸链霉菌酶消化沃纳综合征患者尿液中大分子AGAG的酶法分析。在Sephadex G-100柱上通过凝胶过滤测定了尿AGAG的分子量依赖性分布。在大分子组分中,HA和HS的分布占优势。硫酸软骨素同分异构体在低分子量馏分中表现突出,但随着分子量的减小,4型与6型的比例增加。这些观察结果表明,维尔纳综合征是一种分子量依赖性AGAG组成的代谢性疾病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The molecular weight-dependent distribution of urinary glycosaminoglycans in Werner's syndrome

The macromolecular AGAG in the urine of patients with Werner's syndrome were analyzed by enzymatic methods after digestion with chondroitinases and Streptomyces hyaluronidase. The molecular weight-dependent distribution of the urinary AGAG has been determined by gel filtration on a Sephadex G-100 column.

The distribution of HA and HS was predominant in the macromolecular fractions. Chondroitin sulfate isomers were prominent in the low molecular weight fractions but the ratio of the 4-type to the 6-type increased with decreasing molecular weight. These observations indicated that Werner's syndrome is a metabolic disorder of the molecular weight-dependent AGAG composition.

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