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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
2015
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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 |
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