Classical mitochondrial disease (MD) represents a group of complex metabolic syndromes primarily linked to dysfunction of the mitochondrial ATP-generating oxidative phosphorylation (OXPHOS) system. To date, effective therapies for these diseases are lacking. Here we discuss the ketogenic diet (KD), being a high-fat, moderate protein, and low carbohydrate diet, as a potential intervention strategy. We concisely review the impact of the KD on bioenergetics, ROS/redox metabolism, mitochondrial dynamics and mitophagy. Next, the consequences of the KD in (models of) MD, as well as KD adverse effects, are described. It is concluded that the current experimental evidence suggests that the KD can positively impact on mitochondrial bioenergetics, mitochondrial ROS/redox metabolism and mitochondrial dynamics. However, more information is required on the bioenergetic consequences and mechanistic mode-of-action aspects of the KD at the cellular level and in MD patients.

经典线粒体疾病 (MD) 代表一组复杂的代谢综合征，主要与产生线粒体 ATP 的氧化磷酸化 (OXPHOS) 系统功能障碍有关。迄今为止，缺乏针对这些疾病的有效疗法。在这里，我们讨论生酮饮食 (KD)，它是一种高脂肪、中等蛋白质和低碳水化合物的饮食，作为一种潜在的干预策略。我们简要回顾了 KD 对生物能量学、ROS/氧化还原代谢、线粒体动力学和线粒体自噬的影响。接下来，描述了 MD（模型）中 KD 的后果以及 KD 不利影响。得出的结论是，目前的实验证据表明，KD 可以对线粒体生物能学、线粒体 ROS/氧化还原代谢和线粒体动力学产生积极影响。然而，