{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T20:45:07Z","timestamp":1789764307507,"version":"4.0.1"},"reference-count":57,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2022,8,31]],"date-time":"2022-08-31T00:00:00Z","timestamp":1661904000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Changhua Christian Hospital","award":["104-CCH-ICO-007"],"award-info":[{"award-number":["104-CCH-ICO-007"]}]},{"name":"Changhua Christian Hospital","award":["102-2314-B-371-002"],"award-info":[{"award-number":["102-2314-B-371-002"]}]},{"name":"Changhua Christian Hospital","award":["CSH-2015-C-008"],"award-info":[{"award-number":["CSH-2015-C-008"]}]},{"name":"National Science Council, Taiwan","award":["104-CCH-ICO-007"],"award-info":[{"award-number":["104-CCH-ICO-007"]}]},{"name":"National Science Council, Taiwan","award":["102-2314-B-371-002"],"award-info":[{"award-number":["102-2314-B-371-002"]}]},{"name":"National Science Council, Taiwan","award":["CSH-2015-C-008"],"award-info":[{"award-number":["CSH-2015-C-008"]}]},{"name":"Chung Shan Medical University Hospital","award":["104-CCH-ICO-007"],"award-info":[{"award-number":["104-CCH-ICO-007"]}]},{"name":"Chung Shan Medical University Hospital","award":["102-2314-B-371-002"],"award-info":[{"award-number":["102-2314-B-371-002"]}]},{"name":"Chung Shan Medical University Hospital","award":["CSH-2015-C-008"],"award-info":[{"award-number":["CSH-2015-C-008"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Coenzyme Q10 (CoQ10), a well-known antioxidant, has been explored as a treatment in several neurodegenerative diseases, but its utility in spinocerebellar ataxia type 3 (SCA3) has not been explored. Herein, the protective effect of CoQ10 was examined using a transgenic mouse model of SCA3 onset. These results demonstrated that a diet supplemented with CoQ10 significantly improved murine locomotion, revealed by rotarod and open-field tests, compared with untreated controls. Additionally, a histological analysis showed the stratification of cerebellar layers indistinguishable from that of wild-type littermates. The increased survival of Purkinje cells was reflected by the reduced abundance of TUNEL-positive nuclei and apoptosis markers of activated p53, as well as lower levels of cleaved caspase 3 and cleaved poly-ADP-ribose polymerase. CoQ10 effects were related to the facilitation of the autophagy-mediated clearance of mutant ataxin-3 protein, as evidenced by the increased expression of heat shock protein 27 and autophagic markers p62, Beclin-1 and LC3II. The expression of antioxidant enzymes heme oxygenase 1 (HO-1), glutathione peroxidase 1 (GPx1) and superoxide dismutase 1 (SOD1) and 2 (SOD2), but not of glutathione peroxidase 2 (GPx2), were restored in 84Q SCA3 mice treated with CoQ10 to levels even higher than those measured in wild-type control mice. Furthermore, CoQ10 treatment also prevented skeletal muscle weight loss and muscle atrophy in diseased mice, revealed by significantly increased muscle fiber area and upregulated muscle protein synthesis pathways. In summary, our results demonstrated biochemical and pharmacological bases for the possible use of CoQ10 in SCA3 therapy.<\/jats:p>","DOI":"10.3390\/nu14173593","type":"journal-article","created":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T01:50:51Z","timestamp":1661997051000},"page":"3593","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Coenzyme Q10 Supplementation Increases Removal of the ATXN3 Polyglutamine Repeat, Reducing Cerebellar Degeneration and Improving Motor Dysfunction in Murine Spinocerebellar Ataxia Type 3"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0028-0183","authenticated-orcid":false,"given":"Yu-Ling","family":"Wu","sequence":"first","affiliation":[{"name":"Vascular and Genomic Center, Institute of ATP, Changhua Christian Hospital, Changhua 50091, Taiwan"},{"name":"Cardiovascular and Mitochondrial Related Disease Research Center, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien 97004, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8349-862X","authenticated-orcid":false,"given":"Jui-Chih","family":"Chang","sequence":"additional","affiliation":[{"name":"Center of Regenerative Medicine and Tissue Repair, Changhua Christian Hospital, Changhua 50091, Taiwan"},{"name":"General Research Laboratory of Research Department, Changhua Christian Hospital, Changhua 50091, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hai-Lun","family":"Sun","sequence":"additional","affiliation":[{"name":"School of Medicine, Chung Shan Medical University, Taichung 40203, Taiwan"},{"name":"Department of Pediatrics, Division of Allergy, Asthma and Rheumatology, Chung Shan Medical University Hospital, Taichung 40203, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen-Ling","family":"Cheng","sequence":"additional","affiliation":[{"name":"Vascular and Genomic Center, Institute of ATP, Changhua Christian Hospital, Changhua 50091, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Pei","family":"Yen","sequence":"additional","affiliation":[{"name":"Department of Nutrition, Chung Shan Medical University, Taichung 40203, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong-Shiou","family":"Lin","sequence":"additional","affiliation":[{"name":"Vascular and Genomic Center, Institute of ATP, Changhua Christian Hospital, Changhua 50091, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi-Chun","family":"Chao","sequence":"additional","affiliation":[{"name":"Inflammation Research & Drug Development Center, Changhua Christian Hospital, Changhua 50091, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ko-Hung","family":"Liu","sequence":"additional","affiliation":[{"name":"Inflammation Research & Drug Development Center, Changhua Christian Hospital, Changhua 50091, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7221-2895","authenticated-orcid":false,"given":"Ching-Shan","family":"Huang","sequence":"additional","affiliation":[{"name":"Center of Regenerative Medicine and Tissue Repair, Changhua Christian Hospital, Changhua 50091, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai-Li","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Nutrition, Chung Shan Medical University, Taichung 40203, Taiwan"},{"name":"Department of Nutrition, Chung Shan Medical University Hospital, Taichung 40203, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chin-San","family":"Liu","sequence":"additional","affiliation":[{"name":"Vascular and Genomic Center, Institute of ATP, Changhua Christian Hospital, Changhua 50091, Taiwan"},{"name":"Department of Neurology, Changhua Christian Hospital, Changhua 50094, Taiwan"},{"name":"Graduate Institute of Integrated Medicine College of Chinese Medicine, China Medical University, Taichung 40447, Taiwan"},{"name":"College of Medicine, National Chung Hsing University, Taichung 40227, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1111\/jnc.14541","article-title":"Machado\u2013Joseph disease\/spinocerebellar ataxia type 3: Lessons from disease pathogenesis and clues into therapy","volume":"148","author":"Matos","year":"2019","journal-title":"J. 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