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Lookup NU author(s): Dr Adam Hassani, Professor Rita HorvathORCiD, Professor Patrick Chinnery
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Purpose of review Treatment options for mitochondrial myopathies remain limited despite rapid advances in the understanding of the molecular basis of these conditions. Existing therapies continue to be evaluated and novel treatment strategies are starting to appear on the horizon. Recent findings Exercise training continues to show promise as a method of improving exercise tolerance and enhancing oxidative capacity. Coenzyme Q10 deficiency appears to be a relatively common finding in mitochondrial disorders and is likely to benefit from exogenous supplementation. Large-scale randomized clinical trials to evaluate these treatment options are now underway and this represents one of the most important developments in recent years. Activation of the peroxisome proliferator-activated receptor/peroxisome proliferator-activated receptor-gamma coactivator-1 alpha pathway has been shown to induce mitochondrial biogenesis leading to a delayed onset of myopathy and prolonged lifespan in mouse models. A ketogenic diet has also been found to induce mitochondrial biogenesis in mice with mitochondrial myopathy. Summary Therapeutic trials of exercise training and coenzyme Q10 supplementation should continue to be offered to patients with mitochondrial myopathies pending the results of evaluation in randomized clinical trials. Further investigation of peroxisome proliferator-activated receptor/peroxisome proliferator-activated receptor-g coactivator-1a pathway activation, ketogenic diets and other new strategies is required.
Author(s): Hassani A, Horvath R, Chinnery PF
Publication type: Article
Publication status: Published
Journal: Current Opinion in Neurology
Year: 2010
Volume: 23
Issue: 5
Pages: 459-465
Print publication date: 01/10/2010
ISSN (print): 1350-7540
ISSN (electronic): 1080-8248
Publisher: Lippincott Williams & Wilkins
URL: http://dx.doi.org/10.1097/WCO.0b013e32833d1096
DOI: 10.1097/WCO.0b013e32833d1096
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