{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T09:42:26Z","timestamp":1792057346362,"version":"4.4.0"},"reference-count":10,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,10,9]],"date-time":"2018-10-09T00:00:00Z","timestamp":1539043200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disord Clin Pract"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Disorders related to dysfunction of coenzyme (CoQ<jats:sub>10<\/jats:sub>) metabolism, including AarF domain containing kinase 3 gene (ADCK3) mutations, have received attention due to the potential for response to CoQ<jats:sub>10<\/jats:sub> supplementation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We describe two new cases of neurological syndromes due to ADCK3 mutations that obtained striking benefit from CoQ10, and a third who did not. We also review 20 cases from the literature in which responses to CoQ10 were documented out of all 38 previously reported cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Despite the remarkable responses in some cases with ataxia and movement disorders (myoclonus, dystonia, tremor), overall, we were not able to identify variables that predicted response to CoQ<jats:sub>10<\/jats:sub> supplementation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Based on our experience and data from the literature, we recommend a minimum of 10\u2009mg\/kg\/day of ubiquinone with titration up to 15\u2009mg\/kg\/day, maintained at least for 6 months in order to obtain or exclude potential benefit from therapy.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mdc3.12667","type":"journal-article","created":{"date-parts":[[2018,7,28]],"date-time":"2018-07-28T00:50:03Z","timestamp":1532739003000},"page":"635-639","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["ADCK3\u2010related Coenzyme Q10 Deficiency: A Potentially Treatable Genetic Disease"],"prefix":"10.1002","volume":"5","author":[{"given":"Anna","family":"Chang","sequence":"first","affiliation":[{"name":"Morton and Gloria Shulman Movement Disorders Clinic, Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, UHN, Division of Neurology University of Toronto Toronto Ontario Canada"},{"name":"Department of Neurology Shin Kong Wu Ho\u2010Su Memorial Hospital Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Ruiz\u2010Lopez","sequence":"additional","affiliation":[{"name":"Morton and Gloria Shulman Movement Disorders Clinic, Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, UHN, Division of Neurology University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Slow","sequence":"additional","affiliation":[{"name":"Morton and Gloria Shulman Movement Disorders Clinic, Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, UHN, Division of Neurology University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Tarnopolsky","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Neuromuscular and Neurometabolic Clinic McMaster University Medical Center Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony E.","family":"Lang","sequence":"additional","affiliation":[{"name":"Morton and Gloria Shulman Movement Disorders Clinic, Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, UHN, Division of Neurology University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renato P","family":"Munhoz","sequence":"additional","affiliation":[{"name":"Morton and Gloria Shulman Movement Disorders Clinic, Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital, UHN, Division of Neurology University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,10,9]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/nure.12011"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2011-301258"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/8904_2013_251"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2013-306483"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2007.12.022"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2011.07.016"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-173"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12742"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13003"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.23129"}],"container-title":["Movement Disorders Clinical Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmdc3.12667","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mdc3.12667","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mdc3.12667","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mdc3.12667","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T09:06:44Z","timestamp":1760951204000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mdc3.12667"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10,9]]},"references-count":10,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2018,11]]}},"alternative-id":["10.1002\/mdc3.12667"],"URL":"https:\/\/doi.org\/10.1002\/mdc3.12667","archive":["Portico"],"relation":{},"ISSN":["2330-1619","2330-1619"],"issn-type":[{"value":"2330-1619","type":"print"},{"value":"2330-1619","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,10,9]]},"assertion":[{"value":"2018-06-01","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-07-20","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-10-09","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}