The ALS-linked E102Q mutation in Sigma receptor-1 leads to ER stress-mediated defects in protein homeostasis and dysregulation of RNA-binding proteins

Amyotrophic lateral sclerosis (ALS) is characterized by the selective degeneration of motor neurons (MNs) and their target muscles. Misfolded proteins which often form intracellular aggregates are a pathological hallmark of ALS. Disruption of the functional interplay between protein degradation (ubi...

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Detalles Bibliográficos
Autores principales: Dreser, Alice, Vollrath, Jan Tilmann, Sechi, Antonio, Johann, Sonja, Roos, Andreas, Yamoah, Alfred, Katona, Istvan, Bohlega, Saeed, Wiemuth, Dominik, Tian, Yuemin, Schmidt, Axel, Vervoorts, Jörg, Dohmen, Marc, Beyer, Cordian, Anink, Jasper, Aronica, Eleonora, Troost, Dirk, Weis, Joachim, Goswami, Anand
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2017
Materias:
Original Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5596426/
https://www.ncbi.nlm.nih.gov/pubmed/28622300
http://dx.doi.org/10.1038/cdd.2017.88

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