Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.

doi:10.1074/jbc.M113.492017

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Article

Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.

The Journal of biological chemistry - 30 Aug 2013

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

Abstract excerpt

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 aggregate-prone and form non-amyloid inclusions in the affected nervous tissues, but the role of these proteinaceous...

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PMID: 23867462
DOI: 10.1074/jbc.M113.492017
PMCID: PMC3757190

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