{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,13]],"date-time":"2025-09-13T15:52:07Z","timestamp":1757778727753,"version":"3.37.3"},"reference-count":48,"publisher":"American Physiological Society","issue":"4","funder":[{"DOI":"10.13039\/100000069","name":"HHS | NIH | National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)","doi-asserted-by":"publisher","award":["AR062128"],"award-info":[{"award-number":["AR062128"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"HHS | NIH | National Heart, Lung, and Blood Institute (NHBLI)","doi-asserted-by":"publisher","award":["HL094499","HL126797"],"award-info":[{"award-number":["HL094499","HL126797"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000968","name":"American Heart Association (AHA)","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000968","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Heart and Circulatory Physiology"],"published-print":{"date-parts":[[2017,4,1]]},"abstract":"<jats:p> Mechanisms underlying the development of idiopathic dilated cardiomyopathy (DCM) remain poorly understood. Using transcription factor expression profiling, we identified estrogen-related receptor-\u03b2 (ESRR\u03b2), a member of the nuclear receptor family of transcription factors, as highly expressed in murine hearts and other highly oxidative striated muscle beds. Mice bearing cardiac-specific deletion of ESRR\u03b2 (MHC-ERRB KO) develop DCM and sudden death at ~10 mo of age. Isolated adult cardiomyocytes from the MHC-ERRB KO mice showed an increase in calcium sensitivity and impaired cardiomyocyte contractility, which preceded echocardiographic cardiac remodeling and dysfunction by several months. Histological analyses of myocardial biopsies from patients with various cardiomyopathies revealed that ESRR\u03b2 protein is absent from the nucleus of cardiomyocytes from patients with DCM but not other forms of cardiomyopathy (ischemic, hypertrophic, and arrhythmogenic right ventricular cardiomyopathy). Taken together these observations suggest that ESRR\u03b2 is a critical component in the onset of DCM by affecting contractility and calcium balance. <\/jats:p><jats:p> NEW &amp; NOTEWORTHY Estrogen-related receptor-\u03b2 (ESRR\u03b2) is highly expressed in the heart and cardiac-specific deletion results in the development of a dilated cardiomyopathy (DCM). ESRR\u03b2 is mislocalized in human myocardium samples with DCM, suggesting a possible role for ESRR\u03b2 in the pathogenesis of DCM in humans. <\/jats:p>","DOI":"10.1152\/ajpheart.00446.2016","type":"journal-article","created":{"date-parts":[[2017,1,27]],"date-time":"2017-01-27T22:10:19Z","timestamp":1485555019000},"page":"H662-H671","source":"Crossref","is-referenced-by-count":22,"title":["Development of dilated cardiomyopathy and impaired calcium homeostasis with cardiac-specific deletion of ESRR\u03b2"],"prefix":"10.1152","volume":"312","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8195-9605","authenticated-orcid":false,"given":"Glenn C.","family":"Rowe","sequence":"first","affiliation":[{"name":"Cardiovascular Institute, Department of Medicine, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts;"},{"name":"Division of Cardiovascular Diseases, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angeliki","family":"Asimaki","sequence":"additional","affiliation":[{"name":"Pathology, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Evan L.","family":"Graham","sequence":"additional","affiliation":[{"name":"Cardiovascular Institute, Department of Medicine, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts;"},{"name":"Department of Cell and Molecular Biology, Karolinska Institutet, Stockholm, Sweden;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kimberly D.","family":"Martin","sequence":"additional","affiliation":[{"name":"Department of Epidemiology, University of Alabama at Birmingham, Birmingham, Alabama;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kenneth B.","family":"Margulies","sequence":"additional","affiliation":[{"name":"Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saumya","family":"Das","sequence":"additional","affiliation":[{"name":"Cardiovascular Institute, Department of Medicine, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts;"},{"name":"Division of Cardiology, Department of Medicine, Massachusetts General Hospital, and Harvard Medical School, Boston, Massachusetts;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffery","family":"Saffitz","sequence":"additional","affiliation":[{"name":"Pathology, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zoltan","family":"Arany","sequence":"additional","affiliation":[{"name":"Cardiovascular Institute, Department of Medicine, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts;"},{"name":"Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; 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