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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...
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 |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2022
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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 |
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