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Mutations in CoQ\n                    <jats:sub>10<\/jats:sub>\n                    biosynthetic genes cause primary CoQ\n                    <jats:sub>10<\/jats:sub>\n                    deficiency (PCoQD) and manifest as mitochondrial disorders. It is often stated that PCoQD patients can be treated by oral CoQ\n                    <jats:sub>10<\/jats:sub>\n                    supplementation. To test this, we compiled all studies describing PCoQD patients up to May 2022. We excluded studies with no data on CoQ\n                    <jats:sub>10<\/jats:sub>\n                    treatment, or with insufficient description of effectiveness. Out of 303 PCoQD patients identified, we retained 89 cases, of which 24 reported improvements after CoQ\n                    <jats:sub>10<\/jats:sub>\n                    treatment (27.0%). In five cases, the patient's condition was reported to deteriorate after halting of CoQ\n                    <jats:sub>10<\/jats:sub>\n                    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, CoQ\n                    <jats:sub>10<\/jats:sub>\n                    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 CoQ\n                    <jats:sub>10<\/jats:sub>\n                    for the treatment of any disease or as dietary supplement.\n                  <\/jats:p>","DOI":"10.1111\/jcmm.17488","type":"journal-article","created":{"date-parts":[[2022,8,19]],"date-time":"2022-08-19T20:44:50Z","timestamp":1660941890000},"page":"4635-4644","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":44,"title":["The efficacy of coenzyme\n                    <scp>\n                      Q\n                      <sub>10<\/sub>\n                    <\/scp>\n                    treatment in alleviating the symptoms of primary coenzyme\n                    <scp>\n                      Q\n                      <sub>10<\/sub>\n                    <\/scp>\n                    deficiency: A systematic 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