Cytochemical and biochemical detection of intracellularly accumulated sialyl glycoconjugates in sialidosis and galactosialidosis fibroblasts with Macckia amurensis

被引：9

作者：

Kotani, M

Yamada, H

Sakuraba, H

机构：

[1] Tokyo Metropolitan Inst Med Sci, Tokyo Metropolitan Org Med Res, Dept Clin Genet, Bunkyo Ku, Tokyo 1138613, Japan

[2] Tokyo Metropolitan Komagome Hosp, Dept Pediat, Bunkyo Ku, Tokyo 1138613, Japan

来源：

CLINICA CHIMICA ACTA | 2004年 / 344卷 / 1-2期

基金：

日本学术振兴会; 日本科学技术振兴机构;

关键词：

sialyl glycoconjugates; sialidosis; galactosialidosis; lectin staining; Western blotting; Macckia amurensis;

D O I：

10.1016/j.cccn.2004.02.032

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

Background: To clarify the pathogenesis of and evaluate experimental therapeutic trials for lysosomal diseases, effective tools for the detection of intracellularly accumulated materials are required. Methods: We examined a series of lectins for staining and blotting of the accumulated glycoconjugates in sialidosis and galactosialidosis. Results: Lysosomally accumulated sialyl glycoconjugates were successfully detected in cultured fibroblasts from patients with these diseases by means of staining and blotting with Macckia amurensis (MAM). Conclusions: This procedure is sensitive and easy, and will be useful not only for biochemical and diagnostic analyses, but also for therapeutic evaluation in these diseases. (C) 2004 Elsevier B.V. All rights reserved.

引用

页码：131 / 135

页数：5

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