{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T04:12:26Z","timestamp":1779250346021,"version":"3.51.4"},"reference-count":23,"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> By using cDNA microarray and RT\u2010PCR techniques, we investigated the genome\u2010wide alteration of gene expression in skin fibroblasts from patients with myoclonic epilepsy and ragged\u2010red fibers (MERRF) syndrome. By screening for the genes with altered levels of expression, we first discovered that matrix metalloproteinase 1 (MMP1) was highly induced in the primary culture of skin fibroblasts of a female patient in a four\u2010generation family with MERRF syndrome. This phenomenon was confirmed in skin fibroblasts from three other MERRF patients harboring about 85% of mtDNA with A8344G mutation. A further study revealed that the expression of MMP1 could be further induced by treatment of the skin fibroblasts with 200 \u03bcM hydrogen peroxide (H<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub>) and inhibited by 1 mM N\u2010acetylcysteine. Moreover, the intracellular level of H<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub> in skin fibroblasts of the female MERRF patient was higher than those of the asymptomatic family members and age\u2010matched healthy controls. These findings imply that the increase in the expression of MMP1 may represent one of the responses to the increased oxidative stress in the skin fibroblasts of MERRF patients. We suggest that in affected tissues the oxidative stress\u2010elicited overexpression of MMP1, and probably other matrix metalloproteinases involved in cytoskeleton remodeling, may play an important role in the pathogenesis and progression of mitochondrial encephalomyopathies such as MERRF syndrome.<\/jats:p>","DOI":"10.1196\/annals.1338.006","type":"journal-article","created":{"date-parts":[[2005,6,17]],"date-time":"2005-06-17T21:58:11Z","timestamp":1119045491000},"page":"55-63","source":"Crossref","is-referenced-by-count":30,"title":["Upregulation of Matrix Metalloproteinase 1 and Disruption of Mitochondrial Network in Skin Fibroblasts of Patients with MERRF Syndrome"],"prefix":"10.1111","volume":"1042","author":[{"given":"YI\u2010SHING","family":"MA","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"YIN\u2010CHIU","family":"CHEN","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"CHING\u2010YOU","family":"LU","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"CHUN\u2010YI","family":"LIU","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":"10.1097\/00019052-200310000-00004"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.283.5407.1482"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0065-2423(03)37007-6"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.9.4820"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.31.21491"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0891-5849(96)00404-2"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0027-5107(98)00220-6"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-8981(01)00809-9"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02254978"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1293.014"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.8.1070"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.1.93"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-001-0502-8"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004010100455"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.36.25869"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M100199200"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.2001.280.1.C53"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-2287-2"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(00)01527-1"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.240520306"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)32274-3"},{"key":"e_1_2_6_23_2","doi-asserted-by":"publisher","DOI":"10.1006\/exnr.2000.7333"},{"key":"e_1_2_6_24_2","doi-asserted-by":"publisher","DOI":"10.1002\/cm.10066"}],"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.1338.006","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/pdf\/10.1196\/annals.1338.006","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T10:00:59Z","timestamp":1695636059000},"score":1,"resource":{"primary":{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/10.1196\/annals.1338.006"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,5]]},"references-count":23,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2005,5]]}},"alternative-id":["10.1196\/annals.1338.006"],"URL":"https:\/\/doi.org\/10.1196\/annals.1338.006","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]]}}}