{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T01:41:54Z","timestamp":1790473314425,"version":"4.1.0"},"reference-count":218,"publisher":"Portland Press Ltd.","issue":"13","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2017,7,1]]},"abstract":"<jats:p>Cardiomyopathies represent a heterogeneous group of diseases that negatively affect heart function. Primary cardiomyopathies specifically target the myocardium, and may arise from genetic [hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy\/dysplasia (ARVC\/D), mitochondrial cardiomyopathy] or genetic and acquired [dilated cardiomyopathy (DCM), restrictive cardiomyopathy (RCM)] etiology. Modern genomics has identified mutations that are common in these populations, while in\u00a0vitro and in\u00a0vivo experimentation with these mutations have provided invaluable insight into the molecular mechanisms native to these diseases. For example, increased myosin heavy chain (MHC) binding and ATP utilization lead to the hypercontractile sarcomere in HCM, while abnormal protein\u2013protein interaction and impaired Ca2+ flux underlie the relaxed sarcomere of DCM. Furthermore, expanded access to genetic testing has facilitated identification of potential risk factors that appear through inheritance and manifest sometimes only in the advanced stages of the disease. In this review, we discuss the genetic and molecular abnormalities unique to and shared between these primary cardiomyopathies and discuss some of the important advances made using more traditional basic science experimentation.<\/jats:p>","DOI":"10.1042\/cs20160170","type":"journal-article","created":{"date-parts":[[2017,6,23]],"date-time":"2017-06-23T20:10:23Z","timestamp":1498248623000},"page":"1375-1392","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":62,"title":["Molecular mechanisms in cardiomyopathy"],"prefix":"10.1042","volume":"131","author":[{"given":"Keith","family":"Dadson","sequence":"first","affiliation":[{"name":"Toronto General Research Institute, Toronto, Ontario Canada, 100 College St., M5G 1L7"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ludger","family":"Hauck","sequence":"additional","affiliation":[{"name":"Toronto General Research Institute, Toronto, Ontario Canada, 100 College St., M5G 1L7"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filio","family":"Billia","sequence":"additional","affiliation":[{"name":"Toronto General Research Institute, Toronto, Ontario Canada, 100 College St., M5G 1L7"},{"name":"Division of Cardiology, University Health Network (UHN), 200 Elizabeth St., Toronto, Ontario Canada, M5G 2C4"},{"name":"Heart and Stroke Richard Lewar Centre of Excellence, University of Toronto, Toronto, Ontario Canada 15G 1L7"},{"name":"Institute of Medical Science, University of Toronto, 1 King's College Circle, Toronto, Ontario Canada M5G 1A8"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2017,6,23]]},"reference":[{"key":"2021113010512321100_B1","doi-asserted-by":"publisher","first-page":"841","DOI":"10.1161\/01.CIR.93.5.841","article-title":"Report of the 1995 World Health Organization\/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies","volume":"93","author":"Richardson","year":"1996","journal-title":"Circulation"},{"key":"2021113010512321100_B2","doi-asserted-by":"publisher","first-page":"1807","DOI":"10.1161\/CIRCULATIONAHA.106.174287","article-title":"Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention","volume":"113","author":"Maron","year":"2006","journal-title":"Circulation"},{"key":"2021113010512321100_B3","doi-asserted-by":"publisher","first-page":"102","DOI":"10.1038\/nrcardio.2016.175","article-title":"Grey zones in cardiomyopathies: defining boundaries between genetic and iatrogenic disease","volume":"14","author":"Quarta","year":"2017","journal-title":"Nat. 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