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Does CoQ10 help mitochondrial disease?

Not clearly. CoQ10 is widely offered for mitochondrial disease, but the best trial, 60 days at a high dose in 30 adults, only slightly changed lactate and oxygen use during exercise, with no gain in strength. Grade C. For Kearns-Sayre syndrome there are only case reports. Specialists still suggest trying it, but as an add-on to heart checks, supervised exercise and specialist care, not a replacement.

Last reviewed

How we grade evidenceEditorial policy

What the studies found

  • Grade C. CoQ10: small changes, no clear benefit. In a double-blind crossover trial, 30 adults with mitochondrial disease took 1,200 mg of CoQ10 a day for 60 days. The rise in lactate after cycling was smaller and oxygen use slightly higher, but strength and resting lactate did not change; a Cochrane review judged it without clinically meaningful improvement. (Glover 2010; Pfeffer 2012)
  • Grade C. Creatine: mixed. Grip strength rose in 7 patients in one small crossover trial, but in 15 people with Kearns-Sayre syndrome or a related eye-muscle form, 6 weeks of creatine changed neither muscle energy nor symptoms. A CoQ10, creatine and lipoic acid mix improved lab markers in 16 patients without clear gains in daily life. (Tarnopolsky 1997; Kornblum 2005; Rodriguez 2007)
  • Grade D. Kearns-Sayre syndrome and CoQ10: only case reports. In an uncontrolled report of 5 patients, CoQ10 was followed by better heart tracings and nerve symptoms. (Ogasahara 1986)
  • Grade D. Folinic acid in Kearns-Sayre: a case series. Of 6 patients with low folate in the spinal fluid, 2 improved and 4 kept getting worse; early treatment looked most hopeful. Experts advise it when spinal fluid folate is low or brain scans show white-matter changes. (Quijada-Fraile 2014; Goldstein, GeneReviews)

Who should be careful

  • The Mitochondrial Medicine Society suggests offering CoQ10 to most patients while calling its evidence sparse, and advises starting supplements one at a time (Parikh 2015). Do this with your mitochondrial specialist, not on your own.
  • CoQ10 may interact with warfarin and insulin (NCCIH).
  • Folic acid is not the same as folinic acid. In two case reports, one in Kearns-Sayre syndrome, high-dose folic acid kept spinal fluid folate low until it was stopped (Akiyama 2022).
  • Kearns-Sayre syndrome can cause heart block. Fainting, near-fainting, a very slow or irregular pulse, or new breathlessness needs emergency care (Goldstein, GeneReviews).
  • Tell any surgical team about the diagnosis: people with mitochondrial disease have a higher risk of anesthesia problems (Parikh 2015). If you are pregnant, breastfeeding or under 18, ask a clinician first.

Try first

  • Heart checks. For Kearns-Sayre syndrome, GeneReviews advises an ECG and heart ultrasound every 6 to 12 months and a pacemaker when conduction block appears (Goldstein, GeneReviews).
  • Supervised exercise. The consensus advises endurance plus strength training that starts at low intensity, after heart screening (Parikh 2015); in 18 people with mitochondrial myopathy, combined training lengthened cycling time, with very low certainty (Voet 2019).

Sources

  1. Glover EI, et al. A randomized trial of coenzyme Q10 in mitochondrial disorders. Muscle Nerve. 2010. PubMed 20886510 · doi:10.1002/mus.21758
  2. Pfeffer G, et al. Treatment for mitochondrial disorders. Cochrane Database Syst Rev. 2012. PubMed 22513923 · doi:10.1002/14651858.CD004426.pub3
  3. Tarnopolsky MA, et al. A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathies. Muscle Nerve. 1997. PubMed 9390662
  4. Kornblum C, et al. Creatine has no beneficial effect on skeletal muscle energy metabolism in patients with single mitochondrial DNA deletions: a placebo-controlled, double-blind 31P-MRS crossover study. Eur J Neurol. 2005. PubMed 15804248 · doi:10.1111/j.1468-1331.2004.00970.x
  5. Rodriguez MC, et al. Beneficial effects of creatine, CoQ10, and lipoic acid in mitochondrial disorders. Muscle Nerve. 2007. PubMed 17080429 · doi:10.1002/mus.20688
  6. Ogasahara S, et al. Treatment of Kearns-Sayre syndrome with coenzyme Q10. Neurology. 1986. PubMed 3941783 · doi:10.1212/wnl.36.1.45
  7. Quijada-Fraile P, et al. Follow-up of folinic acid supplementation for patients with cerebral folate deficiency and Kearns-Sayre syndrome. Orphanet J Rare Dis. 2014. PubMed 25539952 · doi:10.1186/s13023-014-0217-2
  8. Parikh S, et al. Diagnosis and management of mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society. Genet Med. 2015. PubMed 25503498 · doi:10.1038/gim.2014.177
  9. Goldstein A, Falk MJ. Single Large-Scale Mitochondrial DNA Deletion Syndromes. GeneReviews (University of Washington, Seattle). 2003, updated 2023. PubMed 20301382
  10. Akiyama T, et al. Folic acid inhibits 5-methyltetrahydrofolate transport across the blood-cerebrospinal fluid barrier: Clinical biochemical data from two cases. JIMD Rep. 2022. PubMed 36341171 · doi:10.1002/jmd2.12321
  11. Voet NB, et al. Strength training and aerobic exercise training for muscle disease. Cochrane Database Syst Rev. 2019. PubMed 31808555 · doi:10.1002/14651858.CD003907.pub5