{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,5]],"date-time":"2026-01-05T15:08:32Z","timestamp":1767625712503},"reference-count":25,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,10,8]],"date-time":"2004-10-08T00:00:00Z","timestamp":1097193600000},"content-version":"vor","delay-in-days":6065,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1988,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Enzymic activities of the respiratory chain and content of immunochemically detectable subunits in NADH\u2010ubiquinone oxidoreductase (Complex I) were measured in mitochondria from the skeletal muscles of 4 patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). The rotenone\u2010sensitive NADH\u2010cytochrome <jats:italic>c<\/jats:italic> reductase activity was extremely decreased, ranging from 0% to 27% of the control value. In all patients, the content of subunits of Complex I was also reduced in parallel with the rotenone\u2010sensitive NADH\u2010cytochrome <jats:italic>c<\/jats:italic> reductase activity. It is suggested that the variation in the degree of deficiency of Complex I subunits could explain the clinical heterogeneity of patients with MELAS.<\/jats:p>","DOI":"10.1002\/ana.410230312","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T17:55:26Z","timestamp":1104602126000},"page":"287-294","source":"Crossref","is-referenced-by-count":75,"title":["Deficiency of subunits of complex I and mitochondrial encephalomyopathy"],"prefix":"10.1002","volume":"23","author":[{"given":"Takashi","family":"Ichiki","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masashi","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Morimitsu","family":"Nishikimi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takayuki","family":"Ozawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masanori","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiro","family":"Wada","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":"10.1002\/ana.410160409"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(87)80158-0"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(86)91061-2"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799670"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-2944(78)90089-3"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)52451-6"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.32.2.415"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)44883-1"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02179151"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0076-6879(78)53006-1"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0076-6879(78)53007-3"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(83)90586-9"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.76.9.4350"},{"key":"e_1_2_1_15_2","first-page":"735","article-title":"Variation in the levels of Complex I subunits among tissues in a patient with mitochondrial encephalomyopathy and renal dysfunction","volume":"14","author":"Tanaka M","year":"1987","journal-title":"Biochem Int"},{"key":"e_1_2_1_16_2","first-page":"183","article-title":"Immunocytochemical demonstration of cytochrome c oxidase and Complex III in tissues from patients with mitochondrial encephalomyopathy","volume":"9","author":"Sato T","year":"1986","journal-title":"Muscle Nerve"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI111484"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(79)90071-6"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/105.3.553"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410170602"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bi.54.070185.005055"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.3764430"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1038\/314592a0"},{"key":"e_1_2_1_24_2","first-page":"183","article-title":"Evidence for molecular heterogeneity in human Complex I deficiency","volume":"9","author":"Shapira AHV","year":"1986","journal-title":"Muscle Nerve"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(83)90618-9"},{"key":"e_1_2_1_26_2","unstructured":"KobayashiM MorishitaH OkajimaK et al.Successful treatment with succinate supplement in a patient with a deficiency of Complex I (NADH\u2010CoQ reductase) (abstract). 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