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RUS

             

ENG

YearIMPACT-FACTOR
2022  1,200
2021  1,540
2020  1,374
2019  1,023
2018  0,932
2017  0,977
2016  0,799
2015  0,662
2014  0,740
2013  0,739
2012  0,637
2011  0,658
2010  0,654
2009  0,570
2008  0,849
2007  0,805
2006  0,330
2005  0,435
2004  0,623
2003  0,567
2002  0,641
2001  0,490
2000  0,477
1999  0,762
1998  0,785
1997  0,507
1996  0,518
1995  0,502
Vol 51(2017) N 3 p. 341-351; DOI 10.1134/S0026893317020091 Full Text

A.D. Efimova1*, R.K. Ovchinnikov1, A.Yu. Roman1,2, A.V. Maltsev1, V.V. Grigoriev1, E.A. Kovrazhkina3, V.I. Skvortsova3

The FUS protein: Physiological functions and a role in amyotrophic lateral sclerosis

1Institute of Physiologically Active Compounds, Russian Academy of Sciences, Chernogolovka, Moscow oblast, 142432 Russia
2Aix-Marseille Université, Inserm, CRO2 UMR_S 911, Faculté de Pharmacie, Marseille, 13385, France
3Pirogov Russian National Research Medical University Moscow Russia

*efimova.a.d@gmail.com
Received - 2016-04-04; Accepted - 2016-05-24

Certain forms of amyotrophic lateral sclerosis (ALS) are associated with an altered compartmentalization of FUS and its aggregation in the cytoplasm of motoneurons. FUS is a DNA/RNA-binding protein that is involved in DNA repair and the regulation of transcription, splicing, RNA transport, and local translation. Two theories have been proposed to explain the mechanism of the pathophysiological process in ALS. The theories attribute degeneration of motor neurons to either loss or gain of FUS function. The review describes the main physiological functions of FUS and considers evidence for each of the theories of ALS pathogenesis.

FUS, RNA metabolism, protein aggregation, neurodegeneration, amyotrophic lateral sclerosis



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