AN INTRODUCTION TO THE MOLECULAR-BASIS OF INHERITED MYELIN DISEASES

被引：18

作者：

MATTHIEU, JM

机构：

[1] Service de Pédiatrie, Centre Hospitalier Universitaire Vaudois, Lausanne

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1993年 / 16卷 / 04期

关键词：

D O I：

10.1007/BF00711904

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The myelin sheath is an extension of a plasma membrane tightly wrapped around axons. It facilitates conduction while conserving space and energy. Myelin is characterized by a high lipid content (80% of dry weight). Most myelin proteins are unique to that structure and some of them are restricted to the central or peripheral nervous system. In this review a few examples of inherited metabolic disorders affecting the oligodendrocyte and/or the Schwann cells are presented. Emphasis is placed on mutations in animals that represent invaluable models for investigating the molecular mechanisms of inherited myelin diseases in humans.

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页码：724 / 732

页数：9

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