{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,30]],"date-time":"2025-09-30T10:07:03Z","timestamp":1759226823966},"reference-count":39,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2003,8,7]],"date-time":"2003-08-07T00:00:00Z","timestamp":1060214400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2003,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>To determine the relationship between myoglobin (Mb) and the defense system against reactive oxygen species in various myopathies, we performed immunohistochemical analyses of Mb and various antioxidant enzymes, including manganese superoxide dismutase (Mn\u2010SOD), copper zinc SOD (CuZn\u2010SOD), catalase (CAT), and glutathione peroxidase (GSH\u2010Px). Biopsied muscle specimens were obtained from patients with chronic progressive external ophthalmoplegia (CPEO), Kearns\u2013Sayre syndrome (KSS), Duchenne muscular dystrophy (DMD), and polymyositis (PM). In patients with CPEO\/KSS, stainings of Mb, SOD, CAT, and GSH\u2010Px in nonatrophic ragged\u2010red fibers (RRFs) were more intense than those in non\u2010RRFs. These pronounced stainings corresponded to ragged\u2010red lesions. The staining intensities of these antioxidant enzymes were significantly correlated with that of Mb (<jats:italic>P<\/jats:italic> &lt; 0.001). Atrophic RRFs in specimens from patients with CPEO\/KSS showed intense stainings of these antioxidant enzymes but not intense staining of Mb. In specimens from patients with DMD\/PM, the antioxidant enzymes but not Mb were overexpressed in degenerative fibers. These results suggest that oxidative stress is associated with Mb expression specifically in mitochondrial diseases. The antioxidant enzymes seem to be upregulated to protect against muscle damage in nonatrophic RRFs. However, the Mb\u2010mediated oxidative damage may become more extensive and result in further mitochondrial dysfunction and progressive atrophy of RRF with impaired upregulation of Mb. Muscle Nerve 28: 484\u2013492, 2003<\/jats:p>","DOI":"10.1002\/mus.10466","type":"journal-article","created":{"date-parts":[[2003,9,19]],"date-time":"2003-09-19T10:39:28Z","timestamp":1063967968000},"page":"484-492","source":"Crossref","is-referenced-by-count":19,"title":["Overexpressions of myoglobin and antioxidant enzymes in ragged\u2010red fibers of skeletal muscle from patients with mitochondrial 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