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Instead, identifying and targeting muscle pathways deregulated in DMD will provide new therapeutic avenues. We report that the expression of nuclear receptor estrogen\u2010related receptor\u2010\u03b3 (ERR\u03b3), and its metabolic and angiogenic targets are downregulated (50\u201385%) in skeletal muscles of mdx mice (DMD model)\n                    <jats:italic>vs.<\/jats:italic>\n                    wild\u2010type mice. Corelatively, oxidative myofibers, muscle vasculature, and exercise tolerance (33%) are decreased in mdx\n                    <jats:italic>vs.<\/jats:italic>\n                    wild\u2010type mice. Overexpressing ERR\u03b3 selectively in the dystrophic muscles of the mdx mice restored metabolic and angiogenic gene expression compared with control mdx mice. Further, ERR\u03b3 enhanced muscle oxidative myofibers, vasculature, and blood flow (by 33\u201366%) and improved exercise tolerance (by 75%) in the dystrophic mice. Restoring muscle ERR\u03b3 pathway ameliorated muscle damage and also prevented DMD hallmarks of postexercise muscle damage, hypoxia, and fatigue in mdx mice. Notably, ERR\u03b3 did not restore sarcolemmal DAG complex, which is thus dispensable for antidystrophic effects of ERR\u03b3. In summary, ERR\u03b3\u2010dependent metabolic and angiogenic gene program is defective in DMD, and we demonstrate that its restoration is a potential strategy for treating muscular dystrophy.\u2014Matsakas, A., Yadav, V., Lorca, S., Narkar, V., Muscle ERR\u03b3 mitigates Duchenne muscular dystrophy\n                    <jats:italic>via<\/jats:italic>\n                    metabolic and angiogenic reprogramming. FASEB J. 27, 4004\u20134016 (2013).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.13-228296","type":"journal-article","created":{"date-parts":[[2013,6,18]],"date-time":"2013-06-18T22:56:50Z","timestamp":1371596210000},"page":"4004-4016","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Muscle ERR\u03b3 mitigates Duchenne muscular dystrophy\n                    <i>via<\/i>\n                    metabolic and angiogenic reprogramming"],"prefix":"10.1096","volume":"27","author":[{"given":"Antonios","family":"Matsakas","sequence":"first","affiliation":[{"name":"Center for Metabolic and Degenerative Diseases Institute of Molecular Medicine University of Texas Medical School at Houston Houston Texas 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