{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T04:50:14Z","timestamp":1773291014828,"version":"3.50.1"},"reference-count":24,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,1,9]],"date-time":"2006-01-09T00:00:00Z","timestamp":1136764800000},"content-version":"vor","delay-in-days":253,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of the New York Academy of Sciences"],"published-print":{"date-parts":[[2005,5]]},"abstract":"<jats:p><jats:bold>A<jats:sc>bstract<\/jats:sc>: <\/jats:bold> Chronic progressive external ophthalmoplegia (CPEO) syndrome is one of the mitochondrial diseases caused by large\u2010scale deletions in mitochondrial DNA (mtDNA) that impair the respiratory function of mitochondria and result in decreased production of ATP in affected tissues. In order to investigate whether CPEO\u2010associated mtDNA mutations (i.e., 4,366\u2010bp and 4,977\u2010 bp large\u2010scale deletions) render human cells more vulnerable to apoptosis, we constructed cybrids carrying the deleted mtDNA. Assays for cell viability, DNA fragmentation, cytochrome <jats:italic>c<\/jats:italic> release, and caspase 3 activation revealed that UV irradiation at 20 J\/m<jats:sup>2<\/jats:sup> triggered apoptosis in all the cybrids. This treatment also produced elevated intracellular levels of reactive oxygen species (ROS). The rate of UV\u2010induced cell death was more pronounced in the cybrids harboring mtDNA deletions than in the control cybrid with wild\u2010type mtDNA. Subsequently, we evaluated the effect of coenzyme Q<jats:sub>10<\/jats:sub> on the UV\u2010triggered apoptosis. The results showed that after pretreatment of the cybrids with 100 \u03bcM coenzyme Q<jats:sub>10<\/jats:sub> the UV\u2010induced cell damage (i.e., ROS production and activation of caspase 3) was significantly reduced. Taken together, these findings suggest that large\u2010scale deletions of mtDNA increased the susceptibility of human cells to the UV\u2010triggered apoptosis and that coenzyme Q<jats:sub>10<\/jats:sub> mitigated the damage; hence, it might potentially serve as a therapeutic agent to treat mitochondrial diseases resulting from mtDNA deletions.<\/jats:p>","DOI":"10.1196\/annals.1338.036","type":"journal-article","created":{"date-parts":[[2005,6,17]],"date-time":"2005-06-17T21:58:11Z","timestamp":1119045491000},"page":"429-438","source":"Crossref","is-referenced-by-count":17,"title":["Attenuation of UV\u2010Induced Apoptosis by Coenzyme Q<sub>10<\/sub> in Human Cells Harboring Large\u2010Scale Deletion of Mitochondrial DNA"],"prefix":"10.1111","volume":"1042","author":[{"given":"CHENG\u2010FENG","family":"LEE","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"CHUN\u2010YI","family":"LIU","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"SHU\u2010MEI","family":"CHEN","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"MARIANNA","family":"SIKORSKA","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"CHEN\u2010YU","family":"LIN","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"TZU\u2010LING","family":"CHEN","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"YAU\u2010HUEI","family":"WEI","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,1,9]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.283.5407.1482"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-291X(05)80136-6"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0169-328X(94)00209-W"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(75)80098-6"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0098-2997(94)90015-9"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.10579"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.2001.tb05640.x"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0781.2000.160501.x"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.2814477"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/22.24.5506"},{"key":"e_1_2_6_12_2","first-page":"391","article-title":"Activation of c\u2010Jun NH2\u2010terminal kinase and subsequent CPP32\/Yama during topoisomerase inhibitor beta\u2010lapachone\u2010induced apoptosis through an oxidation\u2010dependent pathway","volume":"59","author":"Shiah S.G.","year":"1999","journal-title":"Cancer Res."},{"key":"e_1_2_6_13_2","first-page":"29905","article-title":"Cytochrome c\u2010mediated apoptosis in cells lacking mitochondrial DNA","volume":"274","author":"Jiang S.","year":"1999","journal-title":"Signaling pathway involving release and caspase 3 activation is conserved. J. Biol. Chem."},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1042\/bj3480425"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/biof.5520180220"},{"key":"e_1_2_6_16_2","unstructured":"Borowy\u2010borowski M. M. Sikorska & P.R. Walker. 2004. Water\u2010soluble composition of bioactive lipophilic compounds. U.S. Patents 6 045 826 and 6 191 172 B1."},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.1.93"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1293.014"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1080\/10715760290021315"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/biof.5520090224"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.48.5.1238"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1159\/000008130"},{"key":"e_1_2_6_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.taap.2004.04.019"},{"key":"e_1_2_6_24_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.shk.0000048900.46342.37"},{"key":"e_1_2_6_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamem.2003.11.012"}],"container-title":["Annals of the New York Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1196%2Fannals.1338.036","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/pdf\/10.1196\/annals.1338.036","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T10:02:15Z","timestamp":1695636135000},"score":1,"resource":{"primary":{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/10.1196\/annals.1338.036"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,5]]},"references-count":24,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2005,5]]}},"alternative-id":["10.1196\/annals.1338.036"],"URL":"https:\/\/doi.org\/10.1196\/annals.1338.036","archive":["Portico"],"relation":{},"ISSN":["0077-8923","1749-6632"],"issn-type":[{"value":"0077-8923","type":"print"},{"value":"1749-6632","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,5]]}}}