Impaired Mitochondrial Mobility in Charcot-Marie-Tooth Disease

Charcot-Marie-Tooth (CMT) disease is a progressive, peripheral neuropathy and the most commonly inherited neurological disorder. Clinical manifestations of CMT mutations are typically limited to peripheral neurons, the longest cells in the body. Currently, mutations in at least 80 different genes ar...

Full description

Saved in:
Bibliographic Details
Published inFrontiers in cell and developmental biology Vol. 9; p. 624823
Main Authors Schiavon, Cara R, Shadel, Gerald S, Manor, Uri
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 01.02.2021
Subjects
axonal transport deficiency
Cell and Developmental Biology
Charcot-Marie-Tooth (CMT) disease
cytoskeleton
mitochondria
neurodegeneration
organelle transport
neurodegeneration
organelle transport
axonal transport deficiency
cytoskeleton
mitochondria
Charcot-Marie-Tooth (CMT) disease
Online AccessGet full text

Cover

More Information
Summary:Charcot-Marie-Tooth (CMT) disease is a progressive, peripheral neuropathy and the most commonly inherited neurological disorder. Clinical manifestations of CMT mutations are typically limited to peripheral neurons, the longest cells in the body. Currently, mutations in at least 80 different genes are associated with CMT and new mutations are regularly being discovered. A large portion of the proteins mutated in axonal CMT have documented roles in mitochondrial mobility, suggesting that organelle trafficking defects may be a common underlying disease mechanism. This review will focus on the potential role of altered mitochondrial mobility in the pathogenesis of axonal CMT, highlighting the conceptional challenges and potential experimental and therapeutic opportunities presented by this "impaired mobility" model of the disease.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
Reviewed by: Masanori Nakagawa, Kyoto Prefectural University of Medicine, Japan; Roman Polishchuk, Telethon Institute of Genetics and Medicine (TIGEM), Italy
This article was submitted to Membrane Traffic, a section of the journal Frontiers in Cell and Developmental Biology
Edited by: Carlos M. Guardia, National Institutes of Health (NIH), United States
ISSN:2296-634X
2296-634X
DOI:10.3389/fcell.2021.624823