Clinical Trial: The Effects of Exercise Versus Inactivity on People With Mitochondrial Muscle Disease
Study Status: Completed
Recruit Status: Unknown status
Study Type: Interventional
Official Title: Exercise Training and Deconditions: Implications for Therapy in Mitochondrial Myopathy
Brief Summary: Mitochondrial myopathies include various inherited diseases that are caused by damage to the mitochondria, energy-producing structures that fuel the body's processes. The main symptoms are muscle weakness, reduced muscle mass, and difficulty with exercising. The purpose of this study is to determine the effects of exercise training versus inactivity on mitochondrial function in muscle and muscle performance in people with mitochondrial myopathies.
Detailed Summary:
Mitochondrial myopathies are caused by mutant mitochondrial DNA, genetic defects in parts of the mitochondrial DNA. These defects can include missing or deleted DNA that typically codes for certain proteins involved in energy production. These mutations cause individual mitochondria and the body on a whole to produce energy less efficiently. Because muscle cells require extensive energy to function properly, they are particularly impaired by mitochondrial dysfunction. The onset of most mitochondrial myopathies occurs before the age of 20. Initially a person may experience muscle weakness and fatigue during physical activity. Other symptoms may include limited eye mobility, heart arrhythmias, slurred speech, swallowing difficulties, and impaired movement.
There is no cure yet for mitochondrial myopathies, nor is there any adequate treatment to stall disease progression. Exercise, known to boost the production and function of mitochondria in healthy people, may reduce symptoms in people with mitochondrial myopathies by increasing the number and function of normal mitochondria in an individual muscle cell. The purpose of this study is to determine the effects of exercise training versus inactivity on the expression of normal and mutant mitochondrial DNA and on mitochondrial production within muscle cells in people with mitochondrial myopathies. The study will also assess how cell function, physical endurance, heart function, and quality of life are affected by exercise training and inactivity.
Participants in this 2-year study will first undergo physiological exercise testing, magnetic resonance imaging (MRI) of heart and skeletal muscles, a needle biopsy of muscle, and a questionnaire on quality of life. Participants will then be randomly assigned to partake in regular exercise training or no training for 6 months. After 6 m
Sponsor: National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Current Primary Outcome:
- Changes in wild-type (normal), mutant, and total mitochondrial DNA copy number [ Time Frame: Measured at Week 26 ]
- Physiological measure of oxidative metabolism [ Time Frame: Measured at Week 26 ]
Original Primary Outcome:
- Changes in wild-type (normal), mutant, and total mitochondrial DNA copy number measured at Week 26
- physiological measure of oxidative metabolism measured at Week 26
Current Secondary Outcome:
Original Secondary Outcome:
Information By: National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Dates:
Date Received: April 5, 2007
Date Started: June 2007
Date Completion: June 2012
Last Updated: May 18, 2009
Last Verified: May 2009
