| Mitochondrial myopathies: developments in treatment. | |
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MedLine Citation:
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PMID: 20651591 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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-1alpha 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-gamma coactivator-1alpha pathway activation, ketogenic diets and other new strategies is required. |
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Authors:
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Adam Hassani; Rita Horvath; Patrick F Chinnery |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: Current opinion in neurology Volume: 23 ISSN: 1473-6551 ISO Abbreviation: Curr. Opin. Neurol. Publication Date: 2010 Oct |
Date Detail:
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Created Date: 2010-09-02 Completed Date: 2010-12-14 Revised Date: 2013-01-18 |
Medline Journal Info:
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Nlm Unique ID: 9319162 Medline TA: Curr Opin Neurol Country: England |
Other Details:
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Languages: eng Pagination: 459-65 Citation Subset: IM |
Affiliation:
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Mitochondrial Research Group, Institute of Human Genetics, Newcastle University, Newcastle upon Tyne, UK. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Dietary Supplements Exercise Therapy Humans Ketogenic Diet Mitochondrial Myopathies / genetics, pathology, physiopathology, therapy* Mutation PPAR gamma / metabolism Ubiquinone / analogs & derivatives, therapeutic use Vitamins / therapeutic use |
| Grant Support | |
ID/Acronym/Agency:
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//Medical Research Council |
| Chemical | |
Reg. No./Substance:
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0/PPAR gamma; 0/Vitamins; 1339-63-5/Ubiquinone; 303-98-0/coenzyme Q10 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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