ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS

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Through the generation of humanized FUS mice expressing full-length human FUS, we identify that when expressed at near endogenous murine FUS levels, both wild-type and ALS-causing and frontotemporal dementia (FTD)-causing mutations complement the essential function(s) of murine FUS. Replacement of m...

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Detalles Bibliográficos
Autores principales: López-Erauskin, Jone, Tadokoro, Takahiro, Baughn, Michael W., Myers, Brian, McAlonis-Downes, Melissa, Chillon-Marinas, Carlos, Asiaban, Joshua N., Artates, Jonathan, Bui, Anh T., Vetto, Anne P., Lee, Sandra K., Le, Ai Vy, Sun, Ying, Jambeau, Mélanie, Boubaker, Jihane, Swing, Deborah, Qiu, Jinsong, Hicks, Geoffrey G., Ouyang, Zhengyu, Fu, Xiang-Dong, Tessarollo, Lino, Ling, Shuo-Chien, Parone, Philippe A., Shaw, Christopher E., Marsala, Martin, Lagier-Tourenne, Clotilde, Cleveland, Don W., Da Cruz, Sandrine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cell Press 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6277851/
https://www.ncbi.nlm.nih.gov/pubmed/30344044
http://dx.doi.org/10.1016/j.neuron.2018.09.044

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