The ketogenic diet as a therapeutic intervention strategy in mitochondrial disease

Changbo Qu, Jaap Keijer, Merel J.W. Adjobo-Hermans, Melissa van de Wal, Tom Schirris, Clara van Karnebeek, Yihang Pan, Werner J.H. Koopman*

*Corresponding author for this work

Research output: Contribution to journalShort surveyAcademicpeer-review

3 Citations (Scopus)


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.

Original languageEnglish
Article number106050
JournalInternational Journal of Biochemistry and Cell Biology
Publication statusPublished - 21 Jul 2021


  • Bioenergetics
  • Ketone bodies
  • Mitochondria
  • Mitochondrial disease


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