Article

Perspectives of drug-based neuroprotection targeting mitochondria

Revue Neurologique (Impact Factor: 0.66). 05/2014; 170(5). DOI: 10.1016/j.neurol.2014.03.005
Source: PubMed

ABSTRACT

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.

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    • "Mitochondrial disease leading to neurodegeneration is likely, at least on some level, to involve all of these functions [41]. In ND several mitochondrial alterations are found like bioenergetics anomalies in the process of oxidative phosphorylation and ATP production, defects of mitochondrial dynamics, increase sensitivity to apoptosis, and accumulation of damaged mitochondria with unstable mitochondrial DNA [2]. "
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