Mitochondrial myopathies (MM) are caused by mutations that typically affect genes involved in oxidative phosphorylation. Main symptoms are exercise intolerance and fatigue. Currently, there is no specific treatment for MM. Resveratrol (RSV) is a nutritional supplement that in preclinical studies has been shown to stimulate mitochondrial function. We hypothesized that RSV could improve exercise capacity in patients with MM. The study design was randomized, double-blind, cross-over and placebo-controlled. Eleven patients with genetically verified MM were randomized to receive either 1000 mg/day RSV or placebo (P) for 8 weeks followed by a 4-week washout and then the opposite treatment. Primary outcomes were changes in heart rate (HR) during submaximal cycling exercise and peak oxygen utilization (VO₂max) during maximal exercise. Secondary outcomes included reduction in perceived exertion, changes in lactate concentrations, self-rated function (SF-36) and fatigue scores (FSS), activities of electron transport chain complexes I and IV in mononuclear cells and mitochondrial biomarkers in muscle tissue among others. There were no significant differences in primary and secondary outcomes between treatments. Mean HR changes were −0.3 ± 4.3 (RSV) vs 1.8 ± 5.0 bpm (P), P = .241. Mean VO₂max changes were 0.7 ± 1.4 (RSV) vs −0.2 ± 2.3 mL/min/kg (P), P = .203. The study provides evidence that 1000 mg RSV daily is ineffective in improving exercise capacity in adults with MM. These findings indicate that previous in vitro studies suggesting a therapeutic potential for RSV in MM, do not translate into clinically meaningful effects in vivo.

中文翻译：

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白藜芦醇对线粒体肌病患者没有影响：一项交叉随机对照试验

线粒体肌病 (MM) 是由通常影响参与氧化磷酸化的基因的突变引起的。主要症状是运动不耐受和疲劳。目前，没有针对MM的具体治疗方法。白藜芦醇 (RSV) 是一种营养补充剂，临床前研究表明它可以刺激线粒体功能。我们假设 RSV 可以提高 MM 患者的运动能力。研究设计是随机的、双盲的、交叉的和安慰剂对照的。11 名经基因验证的 MM 患者随机接受 1000 mg/天 RSV 或安慰剂 (P) 治疗 8 周，然后进行 4 周冲洗，然后进行相反的治疗。主要结果是次最大自行车运动期间心率 (HR) 的变化和氧气利用峰值 (VO ₂最大）在最大运动期间。次要结果包括感知运动的减少、乳酸浓度的变化、自测功能 (SF-36) 和疲劳评分 (FSS)、单核细胞中电子传递链复合物 I 和 IV 的活性以及肌肉组织中的线粒体生物标志物等。治疗之间的主要和次要结局没有显着差异。平均 HR 变化为 -0.3 ± 4.3 (RSV) vs 1.8 ± 5.0 bpm (P)，P  = .241。平均 VO ₂最大变化为 0.7 ± 1.4 (RSV) 与 -0.2 ± 2.3 mL/min/kg (P)，P  = .203。该研究提供的证据表明，每天 1000 毫克 RSV 对改善患有 MM 的成年人的运动能力无效。这些发现表明，以前的体外研究表明 RSV 在 MM 中具有治疗潜力，但并未转化为体内具有临床意义的效果。