A randomized trial of coenzyme Q10 in mitochondrial disorders

Case reports and open‐label studies suggest that coenzyme Q10 (CoQ10) treatment may have beneficial effects in mitochondrial disease patients; however, controlled trials are warranted to clinically prove its effectiveness. Thirty patients with mitochondrial cytopathy received 1200 mg/day CoQ10 for 60 days in a randomized, double‐blind, cross‐over trial. Blood lactate, urinary markers of oxidative stress, body composition, activities of daily living, quality of life, forearm handgrip strength and oxygen desaturation, cycle exercise cardiorespiratory variables, and brain metabolites were measured. CoQ10 treatment attenuated the rise in lactate after cycle ergometry, increased (∽1.93 ml) VO2/kg lean mass after 5 minutes of cycling (P < 0.005), and decreased gray matter choline‐containing compounds (P < 0.05). Sixty days of moderate‐ to high‐dose CoQ10 treatment had minor effects on cycle exercise aerobic capacity and post‐exercise lactate but did not affect other clinically relevant variables such as strength or resting lactate. Muscle Nerve, 2010