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Utrophin is naturally overexpressed at the sarcolemma of mature dystrophin\u2010deficient fibres in DMD and BMD patients as well as in the\n                    <jats:italic>mdx<\/jats:italic>\n                    Duchenne mouse model. Dystrophin and utrophin can co\u2010localise in human foetal muscle, in the dystrophin\u2010competent fibres from DMD\/BMD carriers, and revertant fibre clusters in biopsies from DMD patients. These findings suggest that utrophin overexpression could act as a surrogate, compensating for the lack of dystrophin, and, as such, it could be used in combination with dystrophin restoration therapies. Different strategies to overexpress utrophin are currently under investigation. In recent years, many compounds have been reported to modulate utrophin expression efficiently in preclinical studies and ameliorate the dystrophic phenotype in animal models of the disease. In this manuscript, we discuss the current knowledge on utrophin protein and the different mechanisms that modulate its expression in skeletal muscle. We also include a comprehensive review of compounds proposed as utrophin regulators and, as such, potential therapeutic candidates for these muscular dystrophies.\n                  <\/jats:p>","DOI":"10.1111\/nan.12735","type":"journal-article","created":{"date-parts":[[2021,5,17]],"date-time":"2021-05-17T10:59:59Z","timestamp":1621249199000},"page":"711-723","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":52,"title":["Utrophin modulator drugs as potential therapies for Duchenne and Becker muscular dystrophies"],"prefix":"10.1111","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7804-8763","authenticated-orcid":false,"given":"Patricia","family":"Soblechero\u2010Mart\u00edn","sequence":"first","affiliation":[{"name":"Neuromuscular Disorders Biocruces Bizkaia Health Research Institute  Barakaldo Spain"},{"name":"Clinical Laboratory Service Osakidetza Basque Health Service Bilbao\u2010Basurto Integrated Health 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