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Emerging Evidence Highlighting the Importance of Redox Dysregulation in the Pathogenesis of Amyotrophic Lateral Sclerosis (ALS)

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journal contribution
posted on 2021-05-04, 00:52 authored by CJ Jagaraj, S Parakh, Julie AtkinJulie Atkin
The cellular redox state, or balance between cellular oxidation and reduction reactions, serves as a vital antioxidant defence system that is linked to all important cellular activities. Redox regulation is therefore a fundamental cellular process for aerobic organisms. Whilst oxidative stress is well described in neurodegenerative disorders including amyotrophic lateral sclerosis (ALS), other aspects of redox dysfunction and their contributions to pathophysiology are only just emerging. ALS is a fatal neurodegenerative disease affecting motor neurons, with few useful treatments. Hence there is an urgent need to develop more effective therapeutics in the future. Here, we discuss the increasing evidence for redox dysregulation as an important and primary contributor to ALS pathogenesis, which is associated with multiple disease mechanisms. Understanding the connection between redox homeostasis, proteins that mediate redox regulation, and disease pathophysiology in ALS, may facilitate a better understanding of disease mechanisms, and lead to the design of better therapeutic strategies.


CJ was supported by a Macquarie University Postgraduate Research Scholarship. This work was supported by the National Health and Medical Research Council of Australia (NHMRC) Project grants (1006141, 10305133, 1086887, and 1095215). Additionally, support was from the Motor Neurone Disease Research Institute of Australia, Angie Cunningham Laugh to Cure MND Grant, Zo-ee Research Grant, Grants in Aid, and the Fight MND Foundation.


Publication Date



Frontiers in Cellular Neuroscience



Article Number

ARTN 581950







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