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C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

The major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis is a G(4)C(2) repeat expansion in C9ORF72. Efforts to combat neurodegeneration associated with “c9FTD/ALS” are hindered by a lack of animal models recapitulating disease features. We developed a mouse model to mimic...

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Pubblicato in:Science
Autori principali: Chew, Jeannie, Gendron, Tania F., Prudencio, Mercedes, Sasaguri, Hiroki, Zhang, Yong-Jie, Castanedes-Casey, Monica, Lee, Chris W., Jansen-West, Karen, Kurti, Aishe, Murray, Melissa E., Bieniek, Kevin F., Bauer, Peter O., Whitelaw, Ena C., Rousseau, Linda, Stankowski, Jeannette N., Stetler, Caroline, Daughrity, Lillian M., Perkerson, Emilie A., Desaro, Pamela, Johnston, Amelia, Overstreet, Karen, Edbauer, Dieter, Rademakers, Rosa, Boylan, Kevin B., Dickson, Dennis W., Fryer, John D., Petrucelli, Leonard
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2015
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4692360/
https://ncbi.nlm.nih.gov/pubmed/25977373
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1126/science.aaa9344
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