通过对排泄的硫酸化粘多糖进行分析来鉴别粘多糖贮积症。
Differentiation of mucopolysaccharidoses by analyses of the excreted sulfated mucopolysaccharides.
作者信息
Cohen D M, Mourão P A, Dietrich C P
出版信息
Clin Chim Acta. 1977 Nov 1;80(3):555-62. doi: 10.1016/0009-8981(77)90150-4.
Analyses of sulfated mucopolysaccharides excreted by patients with Hunter, Hurler, Scheie, Sanfilippo A and Sanfilippo B syndromes are reported. Three distinct methods, namely agarose gel electrophoresis, enzymatic degradation and molecular weight determination, were used in an attempt to differentiate the mucopolysaccharidoses by analysis of the urinary mucopolysaccharides. It is shown that by the combination of these methods it is possible to differentiate most of the syndromes and also to demonstrated that some patients with the same syndrome excrete different types of sulfated mucopolysaccharides.
本文报道了对患有亨特综合征、赫勒综合征、谢伊综合征、桑菲利波A综合征和桑菲利波B综合征患者所排泄的硫酸化粘多糖的分析。为了通过分析尿中的粘多糖来区分粘多糖贮积症，采用了三种不同的方法，即琼脂糖凝胶电泳、酶降解和分子量测定。结果表明，通过这些方法的组合，可以区分大多数综合征，并且还证明了一些患有相同综合征的患者排泄不同类型的硫酸化粘多糖。
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