果蝇肌萎缩侧索硬化症模型。

Drosophila Charcot-Marie-Tooth Disease Models.

机构信息

Department of Applied Biology, Kyoto Institute of Technology, Kyoto, Japan.

Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

出版信息

Adv Exp Med Biol. 2018;1076:97-117. doi: 10.1007/978-981-13-0529-0_7.

DOI:10.1007/978-981-13-0529-0_7

PMID:29951817

Abstract

Charcot-Marie-Tooth disease (CMT) was initially described in 1886. It is characterized by defects in the peripheral nervous system, including sensory and motor neurons. Although more than 80 CMT-causing genes have been identified to date, an effective therapy has not yet been developed for this disease. Since Drosophila does not have axons surrounded by myelin sheaths or Schwann cells, the establishment of a demyelinating CMT model is not appropriate. In this chapter, after overviewing CMT, examples of Drosophila CMT models with axonal neuropathy and other animal CMT models are described.

摘要

Charcot-Marie-Tooth 病（CMT）最初于 1886 年描述。它的特征是外周神经系统（包括感觉和运动神经元）缺陷。尽管迄今为止已经确定了 80 多种导致 CMT 的基因，但这种疾病仍未开发出有效的治疗方法。由于果蝇没有被髓鞘或雪旺细胞包围的轴突，因此不适合建立脱髓鞘 CMT 模型。在本章中，在概述 CMT 之后，描述了具有轴突神经病的果蝇 CMT 模型和其他动物 CMT 模型的例子。