Mol. Cells 2018; 41(9): 818~829  https://doi.org/10.14348/molcells.2018.0243
RNA-Binding Proteins in Amyotrophic Lateral Sclerosis
Melody Zhao1,2,3, Jihye Rachel Kim1,2,3, Rebekah van Bruggen1, and Jeehye Park1,2,*
1Genetics and Genome Biology Program, The Hospital for Sick Children, Toronto, Canada, 2Department of Molecular Genetics, University of Toronto, Toronto, Canada, 3These authors contributed equally to this work.
*Correspondence: jeehye.park@sickkids.ca
Received May 31, 2018; Revised July 23, 2018; Accepted August 10, 2018.; Published online August 29, 2018.
© Korean Society for Molecular and Cellular Biology. All rights reserved.

This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported License. To view a copy of this license, visit (http://creativecommons.org/licenses/by-nc-sa/3.0/).
ABSTRACT
Significant research efforts are ongoing to elucidate the complex molecular mechanisms underlying amyotrophic lateral sclerosis (ALS), which may in turn pinpoint potential therapeutic targets for treatment. The ALS research field has evolved with recent discoveries of numerous genetic mutations in ALS patients, many of which are in genes encoding RNA binding proteins (RBPs), including TDP-43, FUS, ATXN2, TAF15, EWSR1, hnRNPA1, hnRNPA2/B1, MATR3 and TIA1. Accumulating evidence from studies on these ALS-linked RBPs suggests that dysregulation of RNA metabolism, cytoplasmic mislocalization of RBPs, dysfunction in stress granule dynamics of RBPs and increased propensity of mutant RBPs to aggregate may lead to ALS pathogenesis. Here, we review current knowledge of the biological function of these RBPs and the contributions of ALS-linked mutations to disease pathogenesis.
Keywords: ALS, RNA-binding proteins


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