Document Detail


Mitochondrial myopathies: developments in treatment.
MedLine Citation:
PMID:  20651591     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Adam Hassani; Rita Horvath; Patrick F Chinnery
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Current opinion in neurology     Volume:  23     ISSN:  1473-6551     ISO Abbreviation:  Curr. Opin. Neurol.     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-09-02     Completed Date:  2010-12-14     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  9319162     Medline TA:  Curr Opin Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  459-65     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
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:
G0601943//Medical Research Council; //Medical Research Council
Chemical
Reg. No./Substance:
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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