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凝集素组织化学和碳水化合物分析在溶酶体贮积病特征描述中的应用。

Application of lectin histochemistry and carbohydrate analysis to the characterization of lysosomal storage diseases.

作者信息

Alroy J, De Gasperi R, Warren C D

机构信息

Department of Pathology, Tufts University School of Medicine, Boston, Massachusetts 02111.

出版信息

Carbohydr Res. 1991 Jun 25;213:229-50. doi: 10.1016/s0008-6215(00)90611-6.

Abstract

In lysosomal storage diseases that involve a defect in the catabolism of glycoconjugates, lectin histochemistry adds a new dimension to the characterization of stored carbohydrates as it identifies sugar residues in situ in the affected cells and, thus, determines which cell types are affected by storage. It may be combined with chemical and biochemical analysis by h.p.l.c. The present review summarizes recent results for a variety of storage diseases and presents new data for GM1-gangliosidosis.

摘要

在涉及糖缀合物分解代谢缺陷的溶酶体贮积病中,凝集素组织化学为所贮存碳水化合物的特征描述增添了新的维度,因为它能原位识别受影响细胞中的糖残基,从而确定哪些细胞类型受到贮积影响。它可与高效液相色谱法的化学和生化分析相结合。本综述总结了多种贮积病的最新研究结果,并展示了GM1神经节苷脂贮积症的新数据。

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