Table 1.

Treatment options for mitochondrial myopathies

Treatment	Target of intervention	Clinical Trial identifier	Clinical phase	Outcome
CoQ10	Increase respiratory chain flux, serve as antioxidant	NCT00432744	Phase 3	Completed—no data available
Idebenone	Serve as antioxidant	NCT00887562	Phase 2	Primary endpoint did not reach a significant endpoint at the completion of study
		NCT00747487	Phase 2	Improvement in secondary outcome measures in patients with LHON patients with discordant visual acuities
Nicotinamide riboside (NR)		NCT03432871	N/A	Recruiting—no data available.
Acipimox		NCT00943059		2-week treatment, dose of 250 mg three times a day, showed increase in skeletal muscle mitochondrial oxidative capacity. An improvement in the ATP production
Bezafibrate	Mitochondrial biogenesis	NCT02398201	Phase 2	Change in respiratory enzyme activity
RTA-408	NRF2 activator, NFĸB inhibitor, antioxidant	NCT02255422	Phase 2	Completed—no data available
Exercise		NCT00457314	Phase 2	N/A (status of study unknown)—no data available
MTP-131	Cardiolipin stabilization	NCT02367014	Phase 1/2	Improved exercise intolerance and walking distance in 6 min walk test
		NCT02805790	Phase 2	Ongoing
		NCT02976038	Phase 2	Ongoing
KH176	Antioxidant	NCT02909400	Phase 2	Ongoing