RNA-Binding Proteins in Amyotrophic Lateral Sclerosis

Molecules and Cells
Melody ZhaoJeehye Park

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.

Citations

Jan 11, 2020·Frontiers in Neuroscience·Kyle J TrageserGiulio Maria Pasinetti
May 30, 2019·Frontiers in Neurology·Udaya Geetha VijayakumarStephanie Marie-Rose Duguez
Feb 23, 2020·Neurochemical Research·Stephen MooreRita Sattler
Dec 12, 2019·Frontiers in Molecular Biosciences·Kavya Vinayan Pushpalatha, Florence Besse
Aug 7, 2019·Cellular and Molecular Neurobiology·Benedetta PerroneSebastiano Cavallaro
Apr 10, 2021·Frontiers in Cellular Neuroscience·Anna L GillFernando G Vieira
Apr 16, 2021·Frontiers in Neurology·Matt KeonNitin K Saksena

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Methods Mentioned

BETA
immunoprecipitation
RNA-seq
two-hybrid

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