Drosophila Charcot-Marie-Tooth Disease Models

Masamitsu Yamaguchi; Hiroshi Takashima

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

Drosophila Charcot-Marie-Tooth Disease Models

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

Authors

Masamitsu Yamaguchi¹, Hiroshi Takashima²

Affiliations

¹ Department of Applied Biology, Kyoto Institute of Technology, Kyoto, Japan. myamaguc@kit.ac.jp.
² Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

PMID: 29951817
DOI: 10.1007/978-981-13-0529-0_7

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.

Keywords: Charcot-Marie-Tooth disease; Drosophila; Mitochondria; Neuromuscular junction.

Publication types

Review

MeSH terms

Animals
Charcot-Marie-Tooth Disease*
Disease Models, Animal*
Drosophila melanogaster*
Humans