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Fused in Sarcoma (FUS) Protein Lacking Nuclear Localization Signal (NLS) and Major RNA Binding Motifs Triggers Proteinopathy and Severe Motor Phenotype in Transgenic Mice
Dysfunction of two structurally and functionally related proteins, FUS and TAR DNA-binding protein of 43 kDa (TDP-43), implicated in crucial steps of cellular RNA metabolism can cause amyotrophic lateral sclerosis (ALS) and certain other neurodegenerative diseases. The proteins are intrinsically agg...
Autores principales: | Shelkovnikova, Tatyana A., Peters, Owen M., Deykin, Alexey V., Connor-Robson, Natalie, Robinson, Hannah, Ustyugov, Alexey A., Bachurin, Sergey O., Ermolkevich, Tatyana G., Goldman, Igor L., Sadchikova, Elena R., Kovrazhkina, Elena A., Skvortsova, Veronica I., Ling, Shuo-Chien, Da Cruz, Sandrine, Parone, Philippe A., Buchman, Vladimir L., Ninkina, Natalia N. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Society for Biochemistry and Molecular Biology
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3757190/ https://www.ncbi.nlm.nih.gov/pubmed/23867462 http://dx.doi.org/10.1074/jbc.M113.492017 |
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