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Muscle weakness, which was most severe when she was 7 years old, improved gradually with age. Comparison of two muscle biopsies obtained at an interval of 12.5 years (7 and 20 years of age, respectively), revealed that the number of ragged\u2010red fibers was markedly decreased and histochemical cytochrome <jats:italic>c<\/jats:italic> oxidase activity increased in parallel with the decrease in population of mutant genomes.<\/jats:p>","DOI":"10.1002\/ana.410350322","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T14:35:07Z","timestamp":1104590107000},"page":"370-373","source":"Crossref","is-referenced-by-count":21,"title":["Mitochondrial myopathy with progressive decrease in mitochondrial tRNA<sup>Leu(UUR)<\/sup> mutant genomes"],"prefix":"10.1002","volume":"35","author":[{"given":"Yasuhiko","family":"Kawakami","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryoichi","family":"Sakuta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyoshi","family":"Hashimoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Osamu","family":"Fujino","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takehisa","family":"Fujita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masatoshi","family":"Hida","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Horai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yu\u2010ichi","family":"Goto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ikuya","family":"Nonaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,8]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI104637"},{"key":"e_1_2_1_3_2","first-page":"622","article-title":"Mitochondrial diseases","volume":"5","author":"Nonaka I","year":"1992","journal-title":"Curr Opin Neurol Neurosurg"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/348651a0"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410290606"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.42.3.545"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(91)90272-Q"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0892-368"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(92)92560-3"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.41.10.1663"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410140209"},{"key":"e_1_2_1_12_2","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1203\/00006450-199008000-00011","article-title":"Progressive increase of the mutated mitochondrial DNA fraction Kearns\u2010Sayre syndrome","volume":"28","author":"Larsson NG","year":"1990","journal-title":"Pediatr Res"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.23.11164"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.23.10614"},{"key":"e_1_2_1_15_2","first-page":"1187","article-title":"Distribution and threshold expression of the tRNALys mutation in skeletal muscle of patients with myoclonic epilepsy and ragged\u2010red fibers (MERRF)","volume":"51","author":"Boulet L","year":"1992","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(89)92499-6"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.410350322","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.410350322","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T18:36:31Z","timestamp":1695839791000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.410350322"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1994,3]]},"references-count":15,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1994,3]]}},"alternative-id":["10.1002\/ana.410350322"],"URL":"https:\/\/doi.org\/10.1002\/ana.410350322","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[1994,3]]}}}