Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis

The major genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) is a C9orf72 G(4)C(2) repeat expansion(1,2). Proposed mechanisms by which the expansion causes c9FTD/ALS include toxicity from repeat-containing RNA and from dipeptide repeat proteins translated from the...

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Publicat a:Nat Med
Autors principals: Zhang, Yong-Jie, Gendron, Tania F., Ebbert, Mark T. W., O′Raw, Aliesha D., Yue, Mei, Jansen-West, Karen, Zhang, Xu, Prudencio, Mercedes, Chew, Jeannie, Cook, Casey N., Daughrity, Lillian M., Tong, Jimei, Song, Yuping, Pickles, Sarah R., Castanedes-Casey, Monica, Kurti, Aishe, Rademakers, Rosa, Oskarsson, Bjorn, Dickson, Dennis W., Hu, Wenqian, Gitler, Aaron D., Fryer, John D., Petrucelli, Leonard
Format: Artigo
Idioma:Inglês
Publicat: 2018
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6520050/
https://ncbi.nlm.nih.gov/pubmed/29942091
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41591-018-0071-1
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