ALS-causative mutations in FUS/TLS confer gain- and loss-of-function by altered association with SMN and U1-snRNP

The RNA-binding protein FUS/TLS, mutation in which is causative of the fatal motor neuron disease ALS, is demonstrated to directly bind to the U1-snRNP and SMN complexes. ALS-causative mutations in FUS/TLS are shown to abnormally enhance their interaction with SMN and dysregulate its function, inclu...

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Detalles Bibliográficos
Autores principales: Sun, Shuying, Ling, Shuo-Chien, Qiu, Jinsong, Albuquerque, Claudio P., Zhou, Yu, Tokunaga, Seiya, Li, Hairi, Qiu, Haiyan, Bui, Anh, Yeo, Gene W., Huang, Eric J., Eggan, Kevin, Zhou, Huilin, Fu, Xiang-Dong, Lagier-Tourenne, Clotilde, Cleveland, Don W.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2015
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4338613/
https://www.ncbi.nlm.nih.gov/pubmed/25625564
http://dx.doi.org/10.1038/ncomms7171

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