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Expression of human C9ORF72 hexanucleotide expansion reproduces RNA foci and dipeptide repeat proteins but not neurodegeneration in BAC transgenic mice

A non-coding hexanucleotide repeat expansion in the C9ORF72 gene is the most common mutation associated with familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Patients harboring this expansion develop several unique histopathological hallmarks, including intranuclear fo...

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Publié dans:Neuron
Auteurs principaux: Peters, Owen M., Cabrera, Gabriela Toro, Tran, Helene, Gendron, Tania F., McKeon, Jeanne E., Metterville, Jake, Weiss, Alexandra, Wightman, Nicholas, Salameh, Johnny, Kim, Juyhun, Sun, Huaming, Boylan, Kevin B., Dickson, Dennis, Kennedy, Zack, Lin, Ziqiang, Zhang, Yong-Jie, Daughrity, Lillian, Jung, Chris, Gao, Fen-Biao, Sapp, Peter C., Horvitz, H Robert, Bosco, Daryl A., Brown, Solange P., de Jong, Pieter, Petrucelli, Leonard, Mueller, Chris, Brown, Robert H.
Format: Artigo
Langue:Inglês
Publié: 2015
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4828340/
https://ncbi.nlm.nih.gov/pubmed/26637797
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.neuron.2015.11.018
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