A new mouse model of Charcot-Marie-Tooth 2J neuropathy replicates human axonopathy and suggest alteration in axo-glia communication.

A new mouse model of Charcot-Marie-Tooth 2J neuropathy replicates human axonopathy and suggest alteration in axo-glia communication.

Myelin is essential for rapid nerve impulse propagation and axon protection. Accordingly, defects in myelination or myelin maintenance lead to secondary axonal damage and subsequent degeneration. Studies utilizing genetic (CNPase-, MAG-, and PLP-null mice) and naturally occurring neuropathy models s...

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Bibliographic Details
Main Authors: Ghjuvan'Ghjacumu Shackleford, Leandro N Marziali, Yo Sasaki, Anke Claessens, Cinzia Ferri, Nadav I Weinstock, Alexander M Rossor, Nicholas J Silvestri, Emma R Wilson, Edward Hurley, Grahame J Kidd, Senthilvelan Manohar, Dalian Ding, Richard J Salvi, M Laura Feltri, Maurizio D'Antonio, Lawrence Wrabetz
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2022-11-01
Series:PLoS Genetics
Online Access:https://doi.org/10.1371/journal.pgen.1010477

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https://doi.org/10.1371/journal.pgen.1010477

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