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Perk Ablation Ameliorates Myelination in S63del-Charcot–Marie–Tooth 1B Neuropathy

In peripheral nerves, P0 glycoprotein accounts for more than 20% of myelin protein content. P0 is synthesized by Schwann cells, processed in the endoplasmic reticulum (ER) and enters the secretory pathway. However, the mutant P0 with S63 deleted (P0S63del) accumulates in the ER lumen and induces a d...

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Detalles Bibliográficos
Autores principales: Musner, Nicolò, Sidoli, Mariapaola, Zambroni, Desireè, Del Carro, Ubaldo, Ungaro, Daniela, D’Antonio, Maurizio, Feltri, Maria L., Wrabetz, Lawrence
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4844932/
https://www.ncbi.nlm.nih.gov/pubmed/27095827
http://dx.doi.org/10.1177/1759091416642351

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