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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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Detalles Bibliográficos
Autores principales: Shackleford, Ghjuvan’Ghjacumu, Marziali, Leandro N., Sasaki, Yo, Claessens, Anke, Ferri, Cinzia, Weinstock, Nadav I., Rossor, Alexander M., Silvestri, Nicholas J., Wilson, Emma R., Hurley, Edward, Kidd, Grahame J., Manohar, Senthilvelan, Ding, Dalian, Salvi, Richard J., Feltri, M. Laura, D’Antonio, Maurizio, Wrabetz, Lawrence
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9707796/
https://www.ncbi.nlm.nih.gov/pubmed/36350884
http://dx.doi.org/10.1371/journal.pgen.1010477