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Review

The Link between Oxidative Stress, Redox Status, Bioenergetics and Mitochondria in the Pathophysiology of ALS

Department of Physiology, Faculty of Medicine and Odontology, University of Valencia, 15 Av. Blasco Ibañez, 46010 Valencia, Spain
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Authors to whom correspondence should be addressed.
Academic Editors: Melitta Schachner and Kazuhide Hayakawa
Int. J. Mol. Sci. 2021, 22(12), 6352; https://doi.org/10.3390/ijms22126352
Received: 24 April 2021 / Revised: 7 June 2021 / Accepted: 10 June 2021 / Published: 14 June 2021
(This article belongs to the Special Issue Mitochondrial Function in Neurodegenerative Diseases)
Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease of the motor system. It is characterized by the degeneration of both upper and lower motor neurons, which leads to muscle weakness and paralysis. ALS is incurable and has a bleak prognosis, with median survival of 3–5 years after the initial symptomatology. In ALS, motor neurons gradually degenerate and die. Many features of mitochondrial dysfunction are manifested in neurodegenerative diseases, including ALS. Mitochondria have shown to be an early target in ALS pathophysiology and contribute to disease progression. Disruption of their axonal transport, excessive generation of reactive oxygen species, disruption of the mitochondrial structure, dynamics, mitophagy, energy production, calcium buffering and apoptotic triggering have all been directly involved in disease pathogenesis and extensively reported in ALS patients and animal model systems. Alterations in energy production by motor neurons, which severely limit their survival capacity, are tightly linked to the redox status and mitochondria. The present review focuses on this link. Placing oxidative stress as a main pathophysiological mechanism, the molecular interactions and metabolic flows involved are analyzed. This leads to discussing potential therapeutic approaches targeting mitochondrial biology to slow disease progression. View Full-Text
Keywords: ALS; mitochondria; bioenergetics; redox status; oxidative stress ALS; mitochondria; bioenergetics; redox status; oxidative stress
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MDPI and ACS Style

Obrador, E.; Salvador-Palmer, R.; López-Blanch, R.; Jihad-Jebbar, A.; Vallés, S.L.; Estrela, J.M. The Link between Oxidative Stress, Redox Status, Bioenergetics and Mitochondria in the Pathophysiology of ALS. Int. J. Mol. Sci. 2021, 22, 6352. https://doi.org/10.3390/ijms22126352

AMA Style

Obrador E, Salvador-Palmer R, López-Blanch R, Jihad-Jebbar A, Vallés SL, Estrela JM. The Link between Oxidative Stress, Redox Status, Bioenergetics and Mitochondria in the Pathophysiology of ALS. International Journal of Molecular Sciences. 2021; 22(12):6352. https://doi.org/10.3390/ijms22126352

Chicago/Turabian Style

Obrador, Elena, Rosario Salvador-Palmer, Rafael López-Blanch, Ali Jihad-Jebbar, Soraya L. Vallés, and José M. Estrela 2021. "The Link between Oxidative Stress, Redox Status, Bioenergetics and Mitochondria in the Pathophysiology of ALS" International Journal of Molecular Sciences 22, no. 12: 6352. https://doi.org/10.3390/ijms22126352

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