Cytochemical analysis of storage materials in cultured skin fibroblasts from patients with I-cell disease

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• 作者：Ikuo Kawashima ; Mai Ohsawa ; Tomoko Fukushige ; Yoshihisa Nagayama ; Yo Niida ; Masaharu Kotani ; Youichi Tajima ; Takuro Kanekura ; Tamotsu Kanzaki ; Hitoshi Sakuraba
• 关键词：Cytochemistry ; Electronic microscopy ; GM2 gangglioside ; I-cell disease ; Lectin
• 刊名：Clinica Chimica Acta
• 出版年：2007
• 出版时间：March 2007
• 年：2007
• 卷：378
• 期：1-2
• 页码：142-146
• 全文大小：1546 K

文摘

>Background

In cultured fibroblasts from I-cell disease patients the transport of many lysosomal enzymes is defective, and affected cells contain inclusion bodies filled with undegraded substrates. However, the contents of these inclusion bodies have not been well characterized yet. We attempted to identify accumulated substances in cultured I-cell disease fibroblasts cytochemically.

Methods

Cultured fibroblasts from I-cell disease patients were double-stained with a monoclonal antibody to lysosome-associated membrane protein-1 (LAMP-1) and that to GM2 ganglioside, or a series of lectins that specifically bind to sugar moieties.

Results

The patients' cells were granularly stained with the antibody to GM2 ganglioside and the lectins including Maakia amurensis, Datura stramonium, and concanavalin A. Their localization was coincident with that of LAMP-1.

Conclusions

GM2 ganglioside and various kinds of glycoconjugates having sialic acidα2-3galactose, galactoseβ1-4N-acetylglucosamine and mannose residues accumulate in enlarged lysosomes in I-cell disease fibroblasts.