{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,2]],"date-time":"2026-08-02T02:08:22Z","timestamp":1785636502961,"version":"3.56.0"},"reference-count":18,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2004,10,13]],"date-time":"2004-10-13T00:00:00Z","timestamp":1097625600000},"content-version":"vor","delay-in-days":4122,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[1993,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Abnormal carnitine distribution in muscle was found in 22 of 77 patients (29%), with mitochondrial myopathy. Furthermore, total (TC) and free (FC) carnitine levels in muscle were lower in patients than in controls (<jats:italic>P<\/jats:italic> &lt; 0.01). Muscle long\u2010chain acylcarnitines (LCAC) were significantly increased in these patients (<jats:italic>P<\/jats:italic> &lt; 0.01). Muscle carnitine deficiency was found in 31.5% of patients with lipid storage myopathy (LSM) and in 25.6% of patients with ragged\u2010red fibers (RRF). Therefore, carnitine deficiency can be found in patients with mitochondrial myopathy even in the absence of LSM. Muscle levels of TC and FC were lower in patients with respiratory chain defects than in those with normal respiratory chain (<jats:italic>P<\/jats:italic> &lt; 0.01). In contrast, LCAC levels were significantly increased (<jats:italic>P<\/jats:italic> &lt; 0.05). Carnitine levels did not differ significantly, among patients with different respiratory\u2010chain defects. Consequently, these patients, owing to their biochemical block, reduce progressively the muscle carnitine pool and subsequent LCAC rise, due to long\u2010chain fatty acid (LCFA) accumulation.<\/jats:p>","DOI":"10.1002\/mus.880160713","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T08:14:10Z","timestamp":1104567250000},"page":"778-781","source":"Crossref","is-referenced-by-count":31,"title":["Muscle carnitine deficiency and lipid storage myopathy in patients with mitochondrial myopathy"],"prefix":"10.1002","volume":"16","author":[{"given":"Yolanda","family":"Campos","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa","family":"Huertas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Bautista","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Gutierrez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"Aparicio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gustavo","family":"Lorenzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dolores","family":"Segura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes","family":"Villanueva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Cabello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis","family":"Alesso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joaquin","family":"Arenas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,10,13]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bi.57.070188.001401"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880160205"},{"key":"e_1_2_1_4_2","first-page":"45","article-title":"Follow\u2010up and treatment of carnitine deficiencies in man","volume":"7","author":"Di Donato S","year":"1984","journal-title":"Persp Inher Metab Dis"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.36.7.957"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(84)90187-6"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410170602"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.30.8.795"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410220409"},{"key":"e_1_2_1_10_2","first-page":"82","volume-title":"Muscle Biopsy, A Practical approach","author":"Dubowitz V","year":"1973"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.179.4076.899"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF03047372"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.25.1.16"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410240609"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-4165(80)90133-6"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00313387"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(89)90267-2"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.35.7.1041"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880110708"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.880160713","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.880160713","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,24]],"date-time":"2023-10-24T20:02:45Z","timestamp":1698177765000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.880160713"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,7]]},"references-count":18,"journal-issue":{"issue":"7","published-print":{"date-parts":[[1993,7]]}},"alternative-id":["10.1002\/mus.880160713"],"URL":"https:\/\/doi.org\/10.1002\/mus.880160713","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,7]]}}}