The efficacy of coenzyme Q10 treatment in alleviating the symptoms of primary coenzyme Q10 deficiency: A systematic review

source extracted confidence: high 2026-04-09
#ataxia #humans #mitochondrial-diseases #muscle-weakness #ubiquinone

The efficacy of coenzyme Q10 treatment in alleviating the symptoms of primary coenzyme Q10 deficiency: A systematic review

Wang et al., 2022 | J Cell Mol Med | Systematic Review

Citation

Wang Ying, Hekimi Siegfried. The efficacy of coenzyme Q10 treatment in alleviating the symptoms of primary coenzyme Q10 deficiency: A systematic review. J Cell Mol Med. 2022-Sep;26(17):4635-4644. doi:10.1111/jcmm.17488

Abstract

Coenzyme Q10 (CoQ10 ) is necessary for mitochondrial electron transport. Mutations in CoQ10 biosynthetic genes cause primary CoQ10 deficiency (PCoQD) and manifest as mitochondrial disorders. It is often stated that PCoQD patients can be treated by oral CoQ10 supplementation. To test this, we compiled all studies describing PCoQD patients up to May 2022. We excluded studies with no data on CoQ10 treatment, or with insufficient description of effectiveness. Out of 303 PCoQD patients identified, we retained 89 cases, of which 24 reported improvements after CoQ10 treatment (27.0%). In five cases, the patient's condition was reported to deteriorate after halting of CoQ10 treatment. 12 cases reported improvement in the severity of ataxia and 5 cases in the severity of proteinuria. Only a subjective description of improvement was reported for 4 patients described as responding. All reported responses were partial improvements of only some symptoms. For PCoQD patients, CoQ10 supplementation is replacement therapy. Yet, there is only very weak evidence for the efficacy of the treatment. Our findings, thus, suggest a need for caution when seeking to justify the widespread use of CoQ10 for the treatment of any disease or as dietary supplement.

Key Findings

Our findings, thus, suggest a need for caution when seeking to justify the widespread use of CoQ10 for the treatment of any disease or as dietary supplement.

Outcomes Measured

  • Requires manual extraction

Population

Field Value
Population See abstract
Sample Size 4
Age Range See abstract
Condition deficiency

MeSH Terms

  • Ataxia
  • Humans
  • Mitochondrial Diseases
  • Muscle Weakness
  • Ubiquinone

Evidence Classification

  • Level: Systematic Review
  • Publication Types: Journal Article, Systematic Review, Research Support, Non-U.S. Gov't
  • Vertical: coq10

Provenance

Source extracted via PubMed E-utilities API on 2026-04-09