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The role of DNA damage response in amyotrophic lateral sclerosis.

Authors
  • Sun, Yu1, 2
  • Curle, Annabel J1, 2
  • Haider, Arshad M1, 2
  • Balmus, Gabriel1, 2
  • 1 UK Dementia Research Institute at University of Cambridge, Cambridge CB2 0AH, U.K.
  • 2 Department of Clinical Neurosciences, University of Cambridge, Cambridge CB2 0AH, U.K.
Type
Published Article
Journal
Essays in biochemistry
Publication Date
Oct 26, 2020
Volume
64
Issue
5
Pages
847–861
Identifiers
DOI: 10.1042/EBC20200002
PMID: 33078197
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Amyotrophic lateral sclerosis (ALS) is a rapidly disabling and fatal neurodegenerative disease. Due to insufficient disease-modifying treatments, there is an unmet and urgent need for elucidating disease mechanisms that occur early and represent common triggers in both familial and sporadic ALS. Emerging evidence suggests that impaired DNA damage response contributes to age-related somatic accumulation of genomic instability and can trigger or accelerate ALS pathological manifestations. In this review, we summarize and discuss recent studies indicating a direct link between DNA damage response and ALS. Further mechanistic understanding of the role genomic instability is playing in ALS disease pathophysiology will be critical for discovering new therapeutic avenues. © 2020 The Author(s).

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