Journal
JOURNAL OF APPLIED PHYSIOLOGY
Volume 106, Issue 5, Pages 1712-1719Publisher
AMER PHYSIOLOGICAL SOC
DOI: 10.1152/japplphysiol.91571.2008
Keywords
mitochondrial biogenesis; mitochondrial diseases
Categories
Funding
- Muscular Dystrophy Association
- National Institutes of Health [NS-041777, EY-10804]
- United Mitochondrial Disease Foundation
Ask authors/readers for more resources
Wenz T, Diaz F, Hernandez D, Moraes CT. Endurance exercise is protective for mice with mitochondrial myopathy. J Appl Physiol 106: 1712-1719, 2009. First published March 12, 2009; doi:10.1152/japplphysiol.91571.2008.-Defects in the mitochondrial ATP-generating system are one of the most commonly inherited neurological disorders, but they remain without treatment. We have recently shown that modulation of the peroxisome proliferator-activated receptor-gamma coactivator-1 alpha (PGC-1 alpha) level in skeletal muscle of a mitochondrial myopathy mouse model offers a therapeutic approach. Here we analyzed if endurance exercise, which is known to be associated with an increased PGC-1 alpha level in muscle, offers the same beneficial effect. We subjected male and female mice that develop a severe mitochondrial myopathy due to a cytochrome-c oxidase deficiency at 3 mo of age to endurance exercise training and monitored phenotypical and metabolic changes. Sedentary myopathy and wildtype mice were used as controls. Exercise increased PGC-1 alpha in muscle, resulting in increased mitochondrial biogenesis, and successfully stimulated residual respiratory capacity in muscle tissue. As a consequence, ATP levels were increased in exercised mice compared with sedentary myopathy animals, which resulted in a delayed onset of the myopathy and a prolonged lifespan of the exercised mice. As an added benefit, endurance exercise induced antioxidant enzymes. The overall protective effect of endurance exercise delayed the onset of the mitochondrial myopathy and increased life expectancy in the mouse model. Thus stimulating residual oxidative phosphorylation function in the affected muscle by inducing mitochondrial biogenesis through endurance exercise might offer a valuable therapeutic intervention for mitochondrial myopathy patients.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available