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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...

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Autors principals: 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.
Format: Artigo
Idioma:Inglês
Publicat: American Society for Biochemistry and Molecular Biology 2013
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3757190/
https://ncbi.nlm.nih.gov/pubmed/23867462
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M113.492017
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