{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T17:43:05Z","timestamp":1791135785651,"version":"4.1.0"},"reference-count":32,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2007,7,12]],"date-time":"2007-07-12T00:00:00Z","timestamp":1184198400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Traffic"],"published-print":{"date-parts":[[2007,10]]},"abstract":"<jats:p><jats:bold>Muscular dystrophies are a diverse group of severe degenerative muscle diseases. Recent interest in the role of the Golgi complex (GC) in muscle disease has been piqued by findings that several dystrophies result from mutations in putative Golgi\u2010resident glycosyltransferases. Given this new role of the Golgi in sarcolemmal stability, we hypothesized that abnormal Golgi distribution, regulation and\/or function may constitute part of the pathology of other dystrophies, where the primary defect is independent of Golgi function. Thus, we investigated GC organization in the dystrophin\u2010deficient muscles of <jats:italic>mdx <\/jats:italic>mice, a mouse model for Duchenne muscular dystrophy. We report aberrant organization of the synaptic and extrasynaptic GC in skeletal muscles of <jats:italic>mdx <\/jats:italic>mice. The GC is mislocalized and improperly concentrated at the surface and core of <jats:italic>mdx <\/jats:italic>myofibers. Golgi complex localization is disrupted after the onset of necrosis and normal redistribution is impaired during regeneration of <jats:italic>mdx <\/jats:italic>muscle fibers. Disruption of the microtubule cytoskeleton may account in part for aberrant GC localization in <jats:italic>mdx <\/jats:italic>myofibers. Golgi complex distribution is restored to wild type and microtubule cytoskeleton organization is significantly improved by recombinant adeno\u2010associated virus 6\u2010mediated expression of \u0394R4\u2010R23\/\u0394CT microdystrophin showing a novel mode of microdystrophin functionality. In summary, GC distribution abnormalities are a novel component of <jats:italic>mdx <\/jats:italic>skeletal muscle pathology rescued by microdystrophin expression.<\/jats:bold><\/jats:p>","DOI":"10.1111\/j.1600-0854.2007.00622.x","type":"journal-article","created":{"date-parts":[[2007,8,21]],"date-time":"2007-08-21T03:07:18Z","timestamp":1187665638000},"page":"1424-1439","source":"Crossref","is-referenced-by-count":62,"title":["rAAV6\u2010Microdystrophin Rescues Aberrant Golgi Complex Organization in <i>mdx <\/i>Skeletal Muscles"],"prefix":"10.1111","volume":"8","author":[{"given":"Justin M.","family":"Percival","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Gregorevic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guy L.","family":"Odom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glen 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