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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...
Autores principales: | Musner, Nicolò, Sidoli, Mariapaola, Zambroni, Desireè, Del Carro, Ubaldo, Ungaro, Daniela, D’Antonio, Maurizio, Feltri, Maria L., Wrabetz, Lawrence |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2016
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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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