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. 2018 Nov 7;15(4):943–953. doi: 10.1007/s13311-018-00674-4

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© The Author(s) 2018

OpenAccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

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Fig. 3 — Routes to treatment. Schematic depicts the mitochondrial targets of treatments currently being tested for use in treating mitochondrial myopathy. A wide range of approaches are currently in use including the targeting of mitochondrial biogenesis (bezafibrate, RTA-408, exercise, acipimox and nicotinamide riboside), oxidative phosphorylation (CoQ10, idebenone, riboflavin and thiamine), ROS (RTA-498, KH176, CoQ10, idebenone), increasing NAD+ (acipimox and nicotinamide riboside), mtDNA gene editing (mitochondrial TALENS and zinc finger nucleases), lipid utilization (ketogenic diet), and cardiolipin stabilization (elamipretide)