Cargando…
Schwann cells ER-associated degradation contributes to myelin maintenance in adult nerves and limits demyelination in CMT1B mice
In the peripheral nervous system (PNS) myelinating Schwann cells synthesize large amounts of myelin protein zero (P0) glycoprotein, an abundant component of peripheral nerve myelin. In humans, mutations in P0 cause the demyelinating Charcot-Marie-Tooth 1B (CMT1B) neuropathy, one of the most diffused...
Autores principales: | Volpi, Vera G., Ferri, Cinzia, Fregno, Ilaria, Del Carro, Ubaldo, Bianchi, Francesca, Scapin, Cristina, Pettinato, Emanuela, Solda, Tatiana, Feltri, M. Laura, Molinari, Maurizio, Wrabetz, Lawrence, D’Antonio, Maurizio |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6488099/ https://www.ncbi.nlm.nih.gov/pubmed/30995221 http://dx.doi.org/10.1371/journal.pgen.1008069 |
Ejemplares similares
-
Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice
por: D’Antonio, Maurizio, et al.
Publicado: (2013) -
Treatment with IFB-088 Improves Neuropathy in CMT1A and CMT1B Mice
por: Bai, Yunhong, et al.
Publicado: (2022) -
Loss of Fig4 in both Schwann cells and motor neurons contributes to CMT4J neuropathy
por: Vaccari, Ilaria, et al.
Publicado: (2015) -
Perk Ablation Ameliorates Myelination in S63del-Charcot–Marie–Tooth 1B Neuropathy
por: Musner, Nicolò, et al.
Publicado: (2016) -
Disruption of Mtmr2 produces CMT4B1-like neuropathy with myelin outfolding and impaired spermatogenesis
por: Bolino, Alessandra, et al.
Publicado: (2004)