{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T13:57:00Z","timestamp":1777557420494,"version":"3.51.4"},"reference-count":23,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2014,3,27]],"date-time":"2014-03-27T00:00:00Z","timestamp":1395878400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CNS Neurosci Ther"],"published-print":{"date-parts":[[2014,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Aims<\/jats:title><jats:p>The purpose of this study was to evaluate the energy metabolism and mitochondrial function in skeletal muscle from patients with Mitochondrial encephalomyopathy, lactic acidosis, and stroke\u2010like episodes (<jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>) or chronic progressive external ophthalmoplegia (<jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content>) using phosphorus magnetic resonance spectroscopy (<jats:sup>31<\/jats:sup>P\u2010<jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content>), to determine whether abnormally increasing cytochrome c oxidase (<jats:styled-content style=\"fixed-case\">COX<\/jats:styled-content>), as detected in muscle biopsy, could be a cause for <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p><jats:sup>31<\/jats:sup>P\u2010<jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content> was performed on the quadriceps femoris muscle of 12 healthy volunteers and 11 patients diagnosed as <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content> or <jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content> by muscle biopsy and genetic analysis. All subjects experienced a state of rest, 5\u2010min exercise, and 5\u2010min recovery protocol in a supine position.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Compared to <jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> MELAS<\/jats:styled-content> patients typically exhibited <jats:styled-content style=\"fixed-case\">COX<\/jats:styled-content>\u2010positive ragged\u2010red fibers (<jats:styled-content style=\"fixed-case\">RRF<\/jats:styled-content>s) as well as strongly <jats:styled-content style=\"fixed-case\">SDH<\/jats:styled-content>\u2010positive blood vessels (<jats:styled-content style=\"fixed-case\">SSV<\/jats:styled-content>s). However, based on <jats:sup>31<\/jats:sup>P\u2010<jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content> results, <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content> showed a higher inorganic phosphate (Pi)\/phosphocreatine (<jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>r) ratio and lower <jats:styled-content style=\"fixed-case\">ATP<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>r ratio during exercise and delayed Pi\/<jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>r and <jats:styled-content style=\"fixed-case\">ATP<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>r recovery to normal.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study suggests that high <jats:styled-content style=\"fixed-case\">COX<\/jats:styled-content> expression contributes to severe skeletal energy failure by <jats:sup>31<\/jats:sup>P\u2010<jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content> spectroscopy in <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cns.12257","type":"journal-article","created":{"date-parts":[[2014,3,27]],"date-time":"2014-03-27T06:32:50Z","timestamp":1395901970000},"page":"509-514","source":"Crossref","is-referenced-by-count":9,"title":["High Cytochrome c Oxidase Expression Links to Severe Skeletal Energy Failure by <sup>31<\/sup>P\u2010<scp>MRS<\/scp> Spectroscopy in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke\u2010Like Episodes"],"prefix":"10.1111","volume":"20","author":[{"given":"Ai\u2010Hua","family":"Liu","sequence":"first","affiliation":[{"name":"Department of Neurology Drum Tower Hospital of Nanjing Medical University Nanjing 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