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Modifiers of C9orf72 DPR toxicity implicate nucleocytoplasmic transport impairments in c9FTD/ALS

C9orf72 mutations are the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Dipeptide repeat proteins (DPRs) produced by unconventional translation of the C9orf72 repeat expansions cause neurodegeneration in cell culture and in animal models. We performed tw...

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Detalles Bibliográficos
Autores principales: Jovičić, Ana, Mertens, Jerome, Boeynaems, Steven, Bogaert, Elke, Chai, Noori, Yamada, Shizuka B., Paul, Joseph W., Sun, Shuying, Herdy, Joseph R., Bieri, Gregor, Kramer, Nicholas J., Gage, Fred H., Den Bosch, Ludo Van, Robberecht, Wim, Gitler, Aaron D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4552077/
https://www.ncbi.nlm.nih.gov/pubmed/26308983
http://dx.doi.org/10.1038/nn.4085