The role of DNA damage response in amyotrophic lateral sclerosis. Issue 5 (20th October 2020)

• Record Type: Journal Article
• Authors: Sun, Yu; Curle, Annabel J.; Haider, Arshad M.; Balmus, Gabriel
• Editors: Wu, Qian
• Is Part Of: Essays in biochemistry. Volume 64:Issue 5(2020)
• Page Start: 847
• Page End: 861
• Publication Date: 2020-10-20
• Subjects: amyotrophic lateral sclerosis -- DNA damage response -- Frontotemporal dementia -- Genome instability -- Neurodegeneration -- Repeat expansion; Biochemistry -- Periodicals; 572
• DOI: 10.1042/EBC20200002
• Languages: English
• ISSNs: 0071-1365

Abstract

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.