Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice

P0 glycoprotein is an abundant product of terminal differentiation in myelinating Schwann cells. The mutant P0S63del causes Charcot-Marie-Tooth 1B neuropathy in humans, and a very similar demyelinating neuropathy in transgenic mice. P0S63del is retained in the endoplasmic reticulum of Schwann cells,...

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Detalles Bibliográficos
Autores principales: D’Antonio, Maurizio, Musner, Nicolò, Scapin, Cristina, Ungaro, Daniela, Del Carro, Ubaldo, Ron, David, Feltri, M. Laura, Wrabetz, Lawrence
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2013
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3620355/
https://www.ncbi.nlm.nih.gov/pubmed/23547100
http://dx.doi.org/10.1084/jem.20122005

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3620355/
https://www.ncbi.nlm.nih.gov/pubmed/23547100
http://dx.doi.org/10.1084/jem.20122005

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