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). To investigate the pathological role of C9ORF72 in these diseases, we generated a line of mice carrying a bacte...

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Main Authors: Peters, Owen M (Author), Cabrera, Gabriela Toro (Author), Tran, Helene (Author), Gendron, Tania F (Author), McKeon, Jeanne E (Author), Metterville, Jake (Author), Weiss, Alexandra (Author), Wightman, Nicholas (Author), Salameh, Johnny (Author), Kim, Juhyun (Author), Sun, Huaming (Author), Boylan, Kevin B (Author), Dickson, Dennis (Author), Kennedy, Zachary (Author), Lin, Ziqiang (Author), Zhang, Yong-Jie (Author), Daughrity, Lillian (Author), Jung, Chris (Author), Gao, Fen-Biao (Author), Bosco, Daryl A (Author), Brown, Solange P (Author), de Jong, Pieter (Author), Petrucelli, Leonard (Author), Mueller, Christian (Author), Brown, Robert H (Author), Horvitz, Howard Robert (Contributor), Sapp, Peter C. (Author)
Other Authors: Massachusetts Institute of Technology. Department of Biology (Contributor), McGovern Institute for Brain Research at MIT (Contributor), Sapp, Peter C (Contributor)
Format: Article
Language:English
Published: Elsevier, 2016-12-06T20:23:54Z.
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