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In DM1, dominant effects of expanded<jats:italic>CUG<\/jats:italic>repeats result in part from the inactivation of the muscleblind-like (MBNL) proteins. To test the role of MBNL3, we deleted Mbnl3 exon 2 (<jats:italic>Mbnl3<\/jats:italic><jats:sup><jats:italic>\u0394E2<\/jats:italic><\/jats:sup>) in mice and examined the onset of age-associated diseases over 4 to 13 months of age. Accelerated onset of glucose intolerance with elevated insulin levels, cardiac systole deficits, left ventricle hypertrophy, a predictor of a later onset of heart failure and the development of subcapsular and cortical cataracts is observed in<jats:italic>Mbnl3<\/jats:italic><jats:sup><jats:italic>\u0394E2<\/jats:italic><\/jats:sup>mice. Retention of embryonic splice isoforms in adult organs, a prominent defect in DM1, is not observed in multiple RNAs including the Insulin Receptor (<jats:italic>Insr<\/jats:italic>), Cardiac Troponin T (<jats:italic>Tnnt2<\/jats:italic>), Lim Domain Binding 3 (<jats:italic>Ldb3<\/jats:italic>) RNAs in<jats:italic>Mbnl3<\/jats:italic><jats:sup><jats:italic>\u0394E2<\/jats:italic><\/jats:sup>mice. Although rare DM1-like splice errors underlying the observed phenotypes cannot be excluded, our data in conjunction with the reported absence of alternative splice errors in embryonic muscles of a similar<jats:italic>Mbnl3<\/jats:italic><jats:sup><jats:italic>\u0394E2<\/jats:italic><\/jats:sup>mouse by RNA-seq studies, suggest that mechanisms distinct from the adult retention of embryonic splice patterns may make important contributions to the onset of age-associated pathologies in DM1.<\/jats:p>","DOI":"10.1038\/srep30999","type":"journal-article","created":{"date-parts":[[2016,8,3]],"date-time":"2016-08-03T09:48:20Z","timestamp":1470217700000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Muscleblind-like 3 deficit results in a spectrum of age-associated pathologies observed in myotonic dystrophy"],"prefix":"10.1038","volume":"6","author":[{"given":"Jongkyu","family":"Choi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald M.","family":"Dixon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Warunee","family":"Dansithong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walid F.","family":"Abdallah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth P.","family":"Roos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria C.","family":"Jordan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brandon","family":"Trac","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han Shin","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucio","family":"Comai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sita","family":"Reddy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2016,8,3]]},"reference":[{"key":"BFsrep30999_CR1","first-page":"485","volume":"vol. 40","author":"AD Roses","year":"1979","unstructured":"Roses, A. 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