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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2016,11,15]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Defective prelamin A processing causes cardiovascular alterations and premature death in Hutchinson\u2013Gilford progeria syndrome (HGPS) patients and also occurs during physiological aging. We found overt repolarization abnormalities in HGPS patients at advanced disease stages. Similar alterations were present in progeroid\n                    <jats:italic>Zmpste24<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice, which had cardiomyocytes that exhibited prolonged calcium transient duration and reduced sarcoplasmic reticulum calcium loading capacity and release, consistent with absence of isoproterenol-induced ventricular arrhythmias.\n                    <jats:italic>Zmpste24<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice developed age-dependent bradycardia and PQ interval\/QRS complex prolongation, likely contributing to premature death. These defects correlated with mislocalization of connexin43, which was also noted in heart tissue from HGPS patients. These results reveal molecular alterations that might cause cardiac rhythm alterations and premature death in HGPS.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1603754113","type":"journal-article","created":{"date-parts":[[2016,10,31]],"date-time":"2016-10-31T21:59:38Z","timestamp":1477951178000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":46,"title":["Cardiac electrical defects in progeroid mice and Hutchinson\u2013Gilford progeria syndrome patients with nuclear lamina alterations"],"prefix":"10.1073","volume":"113","author":[{"given":"Jos\u00e9","family":"Rivera-Torres","sequence":"first","affiliation":[{"name":"Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), 28029 Madrid, Spain;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Conrado J.","family":"Calvo","sequence":"additional","affiliation":[{"name":"Centro Nacional de Investigaciones Cardiovasculares Carlos III (CNIC), 28029 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