Toggle Main Menu Toggle Search

Open Access padlockePrints

Mitochondrial myopathies: developments in treatment

Lookup NU author(s): Dr Adam Hassani, Professor Rita HorvathORCiD, Professor Patrick Chinnery


Full text for this publication is not currently held within this repository. Alternative links are provided below where available.


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.

Publication metadata

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


DOI: 10.1097/WCO.0b013e32833d1096


Altmetrics provided by Altmetric


Funder referenceFunder name
Association Francaise contre les Myopathies
Medical Research Council (UK)
UK NIHR Biomedical Research Centre for Ageing and Age-related disease
UK Parkinson's Disease Society
BH090164Academy of Medical Sciences, UK
RES0211/7262Newcastle upon Tyne Hospitals NHS Charity