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Because fibrosis and failure of muscle regeneration are the main reasons for the severe disease course of MDC1A, we tested whether L-158809, an analog derivative of losartan, could ameliorate the dystrophy in<jats:italic>dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup><jats:italic>\/dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup>mice, the best-characterized model of MDC1A.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>L-158809 was given in food to<jats:italic>dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup><jats:italic>\/dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup>mice at the age of 3\u2009weeks, and the mice were analyzed at the age of 6 to 7\u2009weeks. We examined the effect of L-158809 on muscle histology and on muscle regeneration after injury as well as the locomotor activity and muscle strength of the mice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found that TGF-\u03b2 signaling in the muscles of the<jats:italic>dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup><jats:italic>\/dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup>mice was strongly increased, and that L-158809 treatment suppressed this signaling. Consequently, L-158809 reduced fibrosis and inflammation in skeletal muscle of<jats:italic>dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup><jats:italic>\/dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup>mice, and largely restored muscle regeneration after toxin-induced injury. Mice showed improvement in their locomotor activity and grip strength, and their body weight was significantly increased.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These data provide evidence that AT1 antagonists ameliorate several hallmarks of MDC1A in<jats:italic>dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup><jats:italic>\/dy<\/jats:italic><jats:sup><jats:italic>W<\/jats:italic><\/jats:sup>mice, the best-characterized mouse model for this disease. Because AT1 antagonists are well tolerated in humans and widely used in clinical practice, these results suggest that losartan may offer a potential future treatment of patients with MDC1A.<\/jats:p><\/jats:sec>","DOI":"10.1186\/2044-5040-2-18","type":"journal-article","created":{"date-parts":[[2012,9,3]],"date-time":"2012-09-03T08:14:35Z","timestamp":1346660075000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Angiotensin II type 1 receptor antagonists alleviate muscle pathology in the mouse model for laminin-\u03b12-deficient congenital muscular dystrophy (MDC1A)"],"prefix":"10.1186","volume":"2","author":[{"given":"Sarina","family":"Meinen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shuo","family":"Lin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Markus A","family":"Ruegg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2012,9,3]]},"reference":[{"key":"62_CR1","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1038\/nm1536","volume":"13","author":"RD Cohn","year":"2007","unstructured":"Cohn RD, van Erp C, Habashi JP, Soleimani AA, Klein EC, Lisi MT, Gamradt M, AP-Rhys CM, Holm TM, Loeys BL, et al.: Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states. 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