Journal
REVUE NEUROLOGIQUE
Volume 170, Issue 5, Pages 390-400Publisher
MASSON EDITEUR
DOI: 10.1016/j.neurol.2014.03.005
Keywords
Mitochondria; Mitochondrial diseases; Neurodegenerative disorders; Pharmacological neuroprotection
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Funding
- Region Pays de la Loire, Association Francaise contre les Myopathies [AFM HAO2013, 17122]
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Mitochondrial dysfunction has been reported in most neurodegenerative diseases. These anomalies include bioenergetic defect, respiratory chain- induced oxidative stress, defects of mitochondrial dynamics, increase sensitivity to apoptosis, and accumulation of damaged mitochondria with instable mitochondrial DNA. Significant progress has been made in our understanding of the pathophysiology of inherited mitochondrial disorders but most have no effective therapies. The development of new metabolic treatments will be useful not only for rare mitochondrial disorders but also for the wide spectrum of common age-related neurodegenerative diseases shown to be associated with mitochondrial dysfunction. A better understanding of the mitochondrial regulating pathways raised several promising perspectives of neuroprotection. This review focuses on the pharmacological approaches to modulate mitochondrial biogenesis, the removal of damaged mitochondria through mitophagy, scavenging free radicals and also dietary measures such as ketogenic diet. (c) 2014 Elsevier Masson SAS. All rights reserved.
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