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Deletion of muscleblind-like 1 (\n                    <jats:italic>Mbnl1<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u0394E2\/\u0394E2<\/jats:italic>\n                    <\/jats:sup>\n                    ) in 129\u2005sv mice results in QRS, QTc widening, bundle block and STc narrowing at 2\u20134 months of age. With time, cardiac function deteriorates further and at 6 months, decreased R wave amplitudes, sinus node dysfunction, cardiac hypertrophy, interstitial fibrosis, multi-focal myocardial fiber death and calcification manifest. Sudden death, where no end point illness is overt, is observed at a median age of 6.5 and 4.8 months in ~67% and ~86% of male and female\n                    <jats:italic>Mbnl1<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u0394E2\/\u0394E2<\/jats:italic>\n                    <\/jats:sup>\n                    mice, respectively. Mbnl1 depletion results in the persistence of embryonic splice isoforms in a network of cardiac RNAs, some of which have been previously implicated in DM1, regulating sodium and calcium currents,\n                    <jats:italic>Scn5a<\/jats:italic>\n                    ,\n                    <jats:italic>Junctin<\/jats:italic>\n                    ,\n                    <jats:italic>Junctate<\/jats:italic>\n                    ,\n                    <jats:italic>Atp2a1<\/jats:italic>\n                    ,\n                    <jats:italic>Atp11a<\/jats:italic>\n                    ,\n                    <jats:italic>Cacna1s<\/jats:italic>\n                    ,\n                    <jats:italic>Ryr2<\/jats:italic>\n                    , intra and inter cellular transport,\n                    <jats:italic>Clta<\/jats:italic>\n                    ,\n                    <jats:italic>Stx2<\/jats:italic>\n                    ,\n                    <jats:italic>Tjp1<\/jats:italic>\n                    , cell survival,\n                    <jats:italic>Capn3<\/jats:italic>\n                    ,\n                    <jats:italic>Sirt2<\/jats:italic>\n                    ,\n                    <jats:italic>Csda<\/jats:italic>\n                    , sarcomere and cytoskeleton organization and function,\n                    <jats:italic>Trim55<\/jats:italic>\n                    ,\n                    <jats:italic>Mapt<\/jats:italic>\n                    ,\n                    <jats:italic>Pdlim3<\/jats:italic>\n                    ,\n                    <jats:italic>Pdlim5<\/jats:italic>\n                    ,\n                    <jats:italic>Sorbs1<\/jats:italic>\n                    ,\n                    <jats:italic>Sorbs2<\/jats:italic>\n                    ,\n                    <jats:italic>Fhod1<\/jats:italic>\n                    ,\n                    <jats:italic>Spag9<\/jats:italic>\n                    and structural components of the sarcomere,\n                    <jats:italic>Myom1<\/jats:italic>\n                    ,\n                    <jats:italic>Tnnt2<\/jats:italic>\n                    ,\n                    <jats:italic>Zasp<\/jats:italic>\n                    . Thus this study supports a key role for Mbnl1 loss in the initiation of DM1 cardiac disease.\n                  <\/jats:p>","DOI":"10.1038\/srep09042","type":"journal-article","created":{"date-parts":[[2015,3,12]],"date-time":"2015-03-12T06:07:07Z","timestamp":1426140427000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":90,"title":["Loss of muscleblind-like 1 results in cardiac pathology and persistence of embryonic splice isoforms"],"prefix":"10.1038","volume":"5","author":[{"given":"Donald M.","family":"Dixon","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jongkyu","family":"Choi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayea","family":"El-Ghazali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sun Young","family":"Park","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":"Michael C.","family":"Fishbein","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":[[2015,3,12]]},"reference":[{"key":"BFsrep09042_CR1","unstructured":"Harper, P. 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