The role of DNA damage response in amyotrophic lateral sclerosis.

DNA damage response Frontotemporal dementia Genome instability Neurodegeneration Repeat expansion amyotrophic lateral sclerosis

Journal

Essays in biochemistry
ISSN: 1744-1358
Titre abrégé: Essays Biochem
Pays: England
ID NLM: 0043306

Informations de publication

Date de publication:
26 10 2020
Historique:
received: 07 07 2020
revised: 28 09 2020
accepted: 29 09 2020
pubmed: 21 10 2020
medline: 8 6 2021
entrez: 20 10 2020
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 33078197
pii: 226703
doi: 10.1042/EBC20200002
pmc: PMC7588667
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

847-861

Subventions

Organisme : Medical Research Council
Pays : United Kingdom

Informations de copyright

© 2020 The Author(s).

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Auteurs

Yu Sun (Y)

UK Dementia Research Institute at University of Cambridge, Cambridge CB2 0AH, U.K.
Department of Clinical Neurosciences, University of Cambridge, Cambridge CB2 0AH, U.K.

Annabel J Curle (AJ)

UK Dementia Research Institute at University of Cambridge, Cambridge CB2 0AH, U.K.
Department of Clinical Neurosciences, University of Cambridge, Cambridge CB2 0AH, U.K.

Arshad M Haider (AM)

UK Dementia Research Institute at University of Cambridge, Cambridge CB2 0AH, U.K.
Department of Clinical Neurosciences, University of Cambridge, Cambridge CB2 0AH, U.K.

Gabriel Balmus (G)

UK Dementia Research Institute at University of Cambridge, Cambridge CB2 0AH, U.K.
Department of Clinical Neurosciences, University of Cambridge, Cambridge CB2 0AH, U.K.

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