期刊
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY
卷 138, 期 -, 页码 -出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.biocel.2021.106050
关键词
Mitochondria; Mitochondrial disease; Ketone bodies; Bioenergetics
资金
- International Exchange Fellowship Program of the Sun Yat-sen University, China
- Research Start-up Fund of the Seventh Affiliated Hospital of Sun Yat-sen University [ZSQYRSFPD0028]
- Sanming Project of Medicine in Shenzhen [SZSM201911003]
The ketogenic diet shows potential positive impacts on mitochondrial bioenergetics, ROS/redox metabolism, and mitochondrial dynamics. However, more research is needed to understand its mechanistic mode of action at the cellular level and in patients with mitochondrial disease.
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.
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