{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T14:13:12Z","timestamp":1781187192832,"version":"3.54.1"},"reference-count":141,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2020,8,31]],"date-time":"2020-08-31T00:00:00Z","timestamp":1598832000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000968","name":"American Heart Association","doi-asserted-by":"publisher","award":["19CDA34660035"],"award-info":[{"award-number":["19CDA34660035"]}],"id":[{"id":"10.13039\/100000968","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005508","name":"Boettcher Foundation","doi-asserted-by":"publisher","award":["25A9520"],"award-info":[{"award-number":["25A9520"]}],"id":[{"id":"10.13039\/100005508","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Research Projects of National Interest from the Ministry of University and Research grant","award":["20173ZWACS"],"award-info":[{"award-number":["20173ZWACS"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Arrhythmogenic cardiomyopathy (ACM) is a heritable myocardial disease that manifests with cardiac arrhythmias, syncope, sudden cardiac death, and heart failure in the advanced stages. The pathological hallmark of ACM is a gradual replacement of the myocardium by fibroadiposis, which typically starts from the epicardium. Molecular genetic studies have identified causal mutations predominantly in genes encoding for desmosomal proteins; however, non-desmosomal causal mutations have also been described, including genes coding for nuclear proteins, cytoskeleton componentsand proteins involved in excitation-contraction coupling. Despite the poor prognosis, currently available treatments can only partially control symptoms and to date there is no effective therapy for ACM. Inhibition of the canonical Wnt\/\u03b2-catenin pathway and activation of the Hippo and the TGF-\u03b2 pathways have been implicated in the pathogenesis of ACM. Yet, our understanding of the molecular mechanisms involved in the development of the disease and the cell source of fibroadiposis remains incomplete. Elucidation of the pathogenesis of the disease could facilitate targeted approaches for treatment. In this manuscript we will provide a comprehensive review of the proposed molecular and cellular mechanisms of the pathogenesis of ACM, including the emerging evidence on abnormal calcium homeostasis and inflammatory\/autoimmune response. Moreover, we will propose novel hypothesis about the role of epicardial cells and paracrine factors in the development of the phenotype. Finally, we will discuss potential innovative therapeutic approaches based on the growing knowledge in the field.<\/jats:p>","DOI":"10.3390\/ijms21176320","type":"journal-article","created":{"date-parts":[[2020,8,31]],"date-time":"2020-08-31T11:53:49Z","timestamp":1598874829000},"page":"6320","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Established and Emerging Mechanisms in the Pathogenesis of Arrhythmogenic Cardiomyopathy: A Multifaceted Disease"],"prefix":"10.3390","volume":"21","author":[{"given":"Shanshan","family":"Gao","sequence":"first","affiliation":[{"name":"Division of Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepa","family":"Puthenvedu","sequence":"additional","affiliation":[{"name":"Division of Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaella","family":"Lombardi","sequence":"additional","affiliation":[{"name":"Division of Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA"},{"name":"Department of Advanced Biomedical Sciences, Federico II University of Naples, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suet Nee","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,8,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1097\/HCO.0b013e3283376daf","article-title":"Arrhythmogenic right ventricular cardiomyopathy is a disease of cardiac stem cells","volume":"25","author":"Lombardi","year":"2010","journal-title":"Curr. Opin. Cardiol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1038\/nrcardio.2011.173","article-title":"Pathophysiology of arrhythmogenic cardiomyopathy","volume":"9","author":"Basso","year":"2011","journal-title":"Nat. Rev. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1093\/eurheartj\/ehq025","article-title":"Diagnosis of arrhythmogenic right ventricular cardiomyopathy\/dysplasia: Proposed modification of the Task Force Criteria","volume":"31","author":"Marcus","year":"2010","journal-title":"Eur. Heart J."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1512","DOI":"10.1016\/S0735-1097(97)00332-X","article-title":"Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy\/dysplasia: A multicenter study","volume":"30","author":"Corrado","year":"1997","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1161\/CIRCGENETICS.109.858217","article-title":"Comprehensive desmosome mutation analysis in north americans with arrhythmogenic right ventricular dysplasia\/cardiomyopathy","volume":"2","author":"Tan","year":"2009","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1093\/europace\/euq104","article-title":"Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia\/cardiomyopathy: Spectrum of mutations and clinical impact in practice","volume":"12","author":"Fressart","year":"2010","journal-title":"Europace"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"41387","DOI":"10.1038\/srep41387","article-title":"Genotype-phenotype relationship in patients with arrhythmogenic right ventricular cardiomyopathy caused by desmosomal gene mutations: A systematic review and meta-analysis","volume":"7","author":"Xu","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1161\/CIRCGENETICS.114.001003","article-title":"Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia\/Cardiomyopathy Patients and Family Members","volume":"8","author":"Groeneweg","year":"2015","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"a029207","DOI":"10.1101\/cshperspect.a029207","article-title":"Adherens Junctions and Desmosomes Coordinate Mechanics and Signaling to Orchestrate Tissue Morphogenesis and Function: An Evolutionary Perspective","volume":"10","author":"Broussard","year":"2018","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.tcm.2009.12.001","article-title":"Cell-cell connection to cardiac disease","volume":"19","author":"Sheikh","year":"2009","journal-title":"Trends Cardiovasc. Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e373","DOI":"10.1016\/j.hrthm.2019.09.019","article-title":"2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: Executive summary","volume":"16","author":"Towbin","year":"2019","journal-title":"Heart Rhythm."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1686","DOI":"10.1016\/j.hrthm.2011.06.026","article-title":"Clinical phenotype and diagnosis of arrhythmogenic right ventricular cardiomyopathy in pediatric patients carrying desmosomal gene mutations","volume":"8","author":"Bauce","year":"2011","journal-title":"Heart Rhythm."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1007\/s10741-019-09830-0","article-title":"Current challenges in sudden cardiac death prevention","volume":"25","author":"Corrado","year":"2020","journal-title":"Heart Fail Rev."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.pcad.2019.04.002","article-title":"Sudden death related cardiomyopathies\u2014Arrhythmogenic right ventricular cardiomyopathy, arrhythmogenic cardiomyopathy, and exercise-induced cardiomyopathy","volume":"62","author":"Goff","year":"2019","journal-title":"Prog. Cardiovasc. Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1056\/NEJMoa1609758","article-title":"Declining Risk of Sudden Death in Heart Failure","volume":"377","author":"Shen","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1221","DOI":"10.1056\/NEJMoa1608029","article-title":"Defibrillator Implantation in Patients with Nonischemic Systolic Heart Failure","volume":"375","author":"Thune","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1161\/CIRCRESAHA.114.302810","article-title":"The hippo pathway is activated and is a causal mechanism for adipogenesis in arrhythmogenic cardiomyopathy","volume":"114","author":"Chen","year":"2014","journal-title":"Circ. Res."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.1161\/CIRCRESAHA.111.255075","article-title":"Nuclear plakoglobin is essential for differentiation of cardiac progenitor cells to adipocytes in arrhythmogenic right ventricular cardiomyopathy","volume":"109","author":"Lombardi","year":"2011","journal-title":"Circ. Res."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2012","DOI":"10.1172\/JCI27751","article-title":"Suppression of canonical Wnt\/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy","volume":"116","author":"Lombardi","year":"2006","journal-title":"J. Clin. Invest."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1093\/cvr\/cvy253","article-title":"A novel murine model for arrhythmogenic cardiomyopathy points to a pathogenic role of Wnt signalling and miRNA dysregulation","volume":"115","author":"Calore","year":"2019","journal-title":"Cardiovasc. Res."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1082","DOI":"10.1093\/cvr\/cvy057","article-title":"Loss of cardiac Wnt\/beta-catenin signalling in desmoplakin-deficient AC8 zebrafish models is rescuable by genetic and pharmacological intervention","volume":"114","author":"Giuliodori","year":"2018","journal-title":"Cardiovasc. Res."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.1128\/MCB.01025-10","article-title":"Cardiac tissue-restricted deletion of plakoglobin results in progressive cardiomyopathy and activation of {beta}-catenin signaling","volume":"31","author":"Li","year":"2011","journal-title":"Mol. Cell Biol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1161\/CIRCULATIONAHA.119.040366","article-title":"Severe Cardiac Dysfunction and Death Caused by Arrhythmogenic Right Ventricular Cardiomyopathy Type 5 Are Improved by Inhibition of Glycogen Synthase Kinase-3beta","volume":"140","year":"2019","journal-title":"Circulation"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1016\/j.hrthm.2012.11.018","article-title":"Remodeling of the cardiac sodium channel, connexin43, and plakoglobin at the intercalated disk in patients with arrhythmogenic cardiomyopathy","volume":"10","author":"Noorman","year":"2013","journal-title":"Heart Rhythm."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"90","DOI":"10.15420\/AER.2016.4.3","article-title":"Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes","volume":"5","author":"Akdis","year":"2016","journal-title":"Arrhythm. Electrophysiol. Rev."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.hrthm.2004.01.001","article-title":"Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease)","volume":"1","author":"Kaplan","year":"2004","journal-title":"Heart Rhythm."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1161\/CIRCRESAHA.109.201418","article-title":"Loss of plakophilin-2 expression leads to decreased sodium current and slower conduction velocity in cultured cardiac myocytes","volume":"105","author":"Sato","year":"2009","journal-title":"Circ. Res."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1093\/cvr\/cvs219","article-title":"Intercalated disc abnormalities, reduced Na(+) current density, and conduction slowing in desmoglein-2 mutant mice prior to cardiomyopathic changes","volume":"95","author":"Rizzo","year":"2012","journal-title":"Cardiovasc. Res."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1161\/CIRCRESAHA.107.154252","article-title":"Connexin43 remodeling caused by inhibition of plakophilin-2 expression in cardiac cells","volume":"101","author":"Oxford","year":"2007","journal-title":"Circ. Res."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.1093\/hmg\/ddt508","article-title":"Connexin defects underlie arrhythmogenic right ventricular cardiomyopathy in a novel mouse model","volume":"23","author":"Lyon","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1038\/s41467-017-00127-0","article-title":"Plakophilin-2 is required for transcription of genes that control calcium cycling and cardiac rhythm","volume":"8","author":"Cerrone","year":"2017","journal-title":"Nat. Commun."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1161\/CIRCULATIONAHA.113.003077","article-title":"Missense mutations in plakophilin-2 cause sodium current deficit and associate with a Brugada syndrome phenotype","volume":"129","author":"Cerrone","year":"2014","journal-title":"Circulation"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1015","DOI":"10.1161\/CIRCULATIONAHA.119.039710","article-title":"Disruption of Ca(2+)i Homeostasis and Connexin 43 Hemichannel Function in the Right Ventricle Precedes Overt Arrhythmogenic Cardiomyopathy in Plakophilin-2-Deficient Mice","volume":"140","author":"Kim","year":"2019","journal-title":"Circulation"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1161\/01.CIR.94.5.983","article-title":"Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis?","volume":"94","author":"Basso","year":"1996","journal-title":"Circulation"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1161\/CIRCULATIONAHA.119.043931","article-title":"Evidence from family studies for autoimmunity in arrhythmogenic right ventricular cardiomyopathy: Associations of circulating anti-heart and anti-intercalated disk autoantibodies with disease severity and family history","volume":"141","author":"Caforio","year":"2020","journal-title":"Circulation"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"3932","DOI":"10.1093\/eurheartj\/ehy567","article-title":"An autoantibody identifies arrhythmogenic right ventricular cardiomyopathy and participates in its pathogenesis","volume":"39","author":"Chatterjee","year":"2018","journal-title":"Eur. Heart J."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1491","DOI":"10.1161\/CIRCULATIONAHA.119.040676","article-title":"Therapeutic Modulation of the Immune Response in Arrhythmogenic Cardiomyopathy","volume":"140","author":"Chelko","year":"2019","journal-title":"Circulation"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"287","DOI":"10.5858\/2000-124-0287-MT","article-title":"Myocyte transdifferentiation: A possible pathogenetic mechanism for arrhythmogenic right ventricular cardiomyopathy","volume":"124","author":"Giordano","year":"2000","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1007\/s00380-008-1079-0","article-title":"Markedly increased intracellular lipid droplets and disruption of intercellular junctions in biopsied myocardium from a patient with arrhythmogenic right ventricular cardiomyopathy","volume":"23","author":"Fujita","year":"2008","journal-title":"Heart Vessels"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.1093\/eurheartj\/ehv579","article-title":"Cardiac mesenchymal stromal cells are a source of adipocytes in arrhythmogenic cardiomyopathy","volume":"37","author":"Sommariva","year":"2016","journal-title":"Eur. Heart J."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1161\/CIRCRESAHA.116.308422","article-title":"Knockdown of Plakophilin 2 downregulates miR-184 through CpG hypermethylation and suppression of the E2F1 pathway and leads to enhanced adipogenesis in vitro","volume":"119","author":"Gurha","year":"2016","journal-title":"Circ. Res."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1161\/CIRCRESAHA.115.308136","article-title":"Cardiac Fibro-Adipocyte Progenitors Express Desmosome Proteins and Preferentially Differentiate to Adipocytes Upon Deletion of the Desmoplakin Gene","volume":"119","author":"Lombardi","year":"2016","journal-title":"Circ. Res."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1161\/CIRCRESAHA.109.196899","article-title":"Genetic fate mapping identifies second heart field progenitor cells as a source of adipocytes in arrhythmogenic right ventricular cardiomyopathy","volume":"104","author":"Lombardi","year":"2009","journal-title":"Circ. Res."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"2119","DOI":"10.1016\/S0140-6736(00)02379-5","article-title":"Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease)","volume":"355","author":"McKoy","year":"2000","journal-title":"Lancet"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"2761","DOI":"10.1093\/hmg\/9.18.2761","article-title":"Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy, woolly hair and keratoderma","volume":"9","author":"Norgett","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1038\/ng1461","article-title":"Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy","volume":"36","author":"Gerull","year":"2004","journal-title":"Nat. Genet."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1650","DOI":"10.1161\/CIRCULATIONAHA.105.609719","article-title":"Plakophilin-2 mutations are the major determinant of familial arrhythmogenic right ventricular dysplasia\/cardiomyopathy","volume":"113","author":"Entius","year":"2006","journal-title":"Circulation"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1226","DOI":"10.1038\/ejhg.2013.39","article-title":"Identification of a PKP2 gene deletion in a family with arrhythmogenic right ventricular cardiomyopathy","volume":"21","author":"Bauce","year":"2013","journal-title":"Eur. J. Hum. Genet."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1200","DOI":"10.1086\/344208","article-title":"Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy","volume":"71","author":"Rampazzo","year":"2002","journal-title":"Am. J. Hum. Genet."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1161\/CIRCULATIONAHA.105.583674","article-title":"Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy","volume":"113","author":"Pilichou","year":"2006","journal-title":"Circulation"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1446","DOI":"10.1016\/j.hrthm.2010.08.007","article-title":"Novel missense mutations in exon 15 of desmoglein-2: Role of the intracellular cadherin segment in arrhythmogenic right ventricular cardiomyopathy?","volume":"7","author":"Gehmlich","year":"2010","journal-title":"Heart Rhythm."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1086\/509044","article-title":"Mutant desmocollin-2 causes arrhythmogenic right ventricular cardiomyopathy","volume":"79","author":"Heuser","year":"2006","journal-title":"Am. J. Hum Genet."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"696.e1","DOI":"10.1016\/j.cjca.2014.01.014","article-title":"Arrhythmogenic right ventricular cardiomyopathy with recessive inheritance related to a new homozygous desmocollin-2 mutation","volume":"30","author":"Krahn","year":"2014","journal-title":"Can. J. Cardiol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1086\/521633","article-title":"A novel dominant mutation in plakoglobin causes arrhythmogenic right ventricular cardiomyopathy","volume":"81","author":"Asimaki","year":"2007","journal-title":"Am. J. Hum. Genet."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/j.cardiores.2004.10.005","article-title":"Regulatory mutations in transforming growth factor-beta3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1","volume":"65","author":"Beffagna","year":"2005","journal-title":"Cardiovasc. Res."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1093\/hmg\/10.3.189","article-title":"Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2)","volume":"10","author":"Tiso","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/j.cardiores.2006.06.008","article-title":"Cardiomyopathies and sudden cardiac death caused by RyR2 mutations: Are the channels the beginning and the end?","volume":"71","author":"Jona","year":"2006","journal-title":"Cardiovasc. Res."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1007\/s12471-013-0413-z","article-title":"The phospholamban p.Arg14del founder mutation in Dutch patients with arrhythmogenic cardiomyopathy","volume":"21","author":"Hassink","year":"2013","journal-title":"Neth Heart J."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1199","DOI":"10.1093\/eurjhf\/hfs119","article-title":"Phospholamban R14del mutation in patients diagnosed with dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy: Evidence supporting the concept of arrhythmogenic cardiomyopathy","volume":"14","author":"Asimaki","year":"2012","journal-title":"Eur. J. Heart Fail."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1016\/j.ajhg.2008.01.010","article-title":"Arrhythmogenic right ventricular cardiomyopathy type 5 is a fully penetrant, lethal arrhythmic disorder caused by a missense mutation in the TMEM43 gene","volume":"82","author":"Merner","year":"2008","journal-title":"Am. J. Hum Genet."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1128","DOI":"10.1093\/eurheartj\/ehr451","article-title":"Mutations in the Lamin A\/C gene mimic arrhythmogenic right ventricular cardiomyopathy","volume":"33","author":"Quarta","year":"2012","journal-title":"Eur. Heart J."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1574","DOI":"10.1016\/j.hrthm.2009.07.041","article-title":"Severe cardiac phenotype with right ventricular predominance in a large cohort of patients with a single missense mutation in the DES gene","volume":"6","author":"Asimaki","year":"2009","journal-title":"Heart Rhythm."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"4595","DOI":"10.1093\/hmg\/ddq387","article-title":"De novo desmin-mutation N116S is associated with arrhythmogenic right ventricular cardiomyopathy","volume":"19","author":"Klauke","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"876","DOI":"10.1161\/CIRCULATIONAHA.110.005405","article-title":"Genetic variation in titin in arrhythmogenic right ventricular cardiomyopathy-overlap syndromes","volume":"124","author":"Taylor","year":"2011","journal-title":"Circulation"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1136\/jmedgenet-2014-102591","article-title":"Titin and desmosomal genes in the natural history of arrhythmogenic right ventricular cardiomyopathy","volume":"51","author":"Brun","year":"2014","journal-title":"J. Med. Genet."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1093\/cvr\/cvw234","article-title":"Multilevel analyses of SCN5A mutations in arrhythmogenic right ventricular dysplasia\/cardiomyopathy suggest non-canonical mechanisms for disease pathogenesis","volume":"113","author":"James","year":"2017","journal-title":"Cardiovasc. Res."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"e001605","DOI":"10.1161\/CIRCGENETICS.116.001605","article-title":"Identification of Cadherin 2 (CDH2) Mutations in Arrhythmogenic Right Ventricular Cardiomyopathy","volume":"10","author":"Mayosi","year":"2017","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1111\/chd.12462","article-title":"Whole exome sequencing with genomic triangulation implicates CDH2-encoded N-cadherin as a novel pathogenic substrate for arrhythmogenic cardiomyopathy","volume":"12","author":"Turkowski","year":"2017","journal-title":"Congenit. Heart Dis."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1093\/eurheartj\/ehs373","article-title":"Mutations in the area composita protein alphaT-catenin are associated with arrhythmogenic right ventricular cardiomyopathy","volume":"34","author":"Calore","year":"2013","journal-title":"Eur. Heart J."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"2440","DOI":"10.1016\/j.jacc.2016.09.927","article-title":"Truncating FLNC Mutations Are Associated With High-Risk Dilated and Arrhythmogenic Cardiomyopathies","volume":"68","author":"Cuenca","year":"2016","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.ijcard.2019.09.048","article-title":"Filamin C variants are associated with a distinctive clinical and immunohistochemical arrhythmogenic cardiomyopathy phenotype","volume":"307","author":"Hall","year":"2020","journal-title":"Int. J. Cardiol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1007\/s12471-013-0401-3","article-title":"Recurrent and founder mutations in the Netherlands-Phospholamban p.Arg14del mutation causes arrhythmogenic cardiomyopathy","volume":"21","author":"Nannenberg","year":"2013","journal-title":"Neth Heart J."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1093\/eurheartj\/ehr040","article-title":"Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: Results from a 10-year registry","volume":"32","author":"Pinamonti","year":"2011","journal-title":"Eur. Heart J."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"2175","DOI":"10.1016\/j.jacc.2008.09.019","article-title":"Left-dominant arrhythmogenic cardiomyopathy: An under-recognized clinical entity","volume":"52","author":"Syrris","year":"2008","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1710","DOI":"10.1161\/CIRCULATIONAHA.106.660241","article-title":"Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia\/cardiomyopathy provides novel insights into patterns of disease expression","volume":"115","author":"Syrris","year":"2007","journal-title":"Circulation"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"e002149","DOI":"10.1161\/JAHA.115.002149","article-title":"Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life-Threatening Arrhythmias","volume":"4","author":"Spezzacatene","year":"2015","journal-title":"J. Am. Heart Assoc."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.hlc.2018.09.010","article-title":"Arrhythmic Genotypes in Familial Dilated Cardiomyopathy: Implications for Genetic Testing and Clinical Management","volume":"28","author":"Peters","year":"2019","journal-title":"Heart Lung. Circ."},{"key":"ref_78","doi-asserted-by":"crossref","unstructured":"Zegkos, T., Panagiotidis, T., Parcharidou, D., and Efthimiadis, G. (2020). Emerging concepts in arrhythmogenic dilated cardiomyopathy. Heart Fail Rev.","DOI":"10.1007\/s10741-020-09933-z"},{"key":"ref_79","doi-asserted-by":"crossref","unstructured":"Corrado, D., Perazzolo Marra, M., Zorzi, A., Beffagna, G., Cipriani, A., Lazzari, M., Migliore, F., Pilichou, K., Rampazzo, A., and Rigato, I. (2020). Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria. Int. J. Cardiol.","DOI":"10.1016\/j.ijcard.2020.06.005"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"1999","DOI":"10.1016\/j.hrthm.2014.07.020","article-title":"Prevalence and significance of rare RYR2 variants in arrhythmogenic right ventricular cardiomyopathy\/dysplasia: Results of a systematic screening","volume":"11","author":"Gandjbakhch","year":"2014","journal-title":"Heart Rhythm."},{"key":"ref_81","doi-asserted-by":"crossref","unstructured":"Van Opbergen, C.J.M., Noorman, M., Pfenniger, A., Copier, J.S., Vermij, S.H., Li, Z., van der Nagel, R., Zhang, M., de Bakker, J.M.T., and Glass, A.M. (2019). Plakophilin-2 Haploinsufficiency Causes Calcium Handling Deficits and Modulates the Cardiac Response Towards Stress. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20174076"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1161\/CIRCRESAHA.110.223412","article-title":"The cardiac desmosome and arrhythmogenic cardiomyopathies: From gene to disease","volume":"107","author":"Delmar","year":"2010","journal-title":"Circ. Res."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1016\/j.ceb.2009.07.002","article-title":"Plakophilins: Multifunctional scaffolds for adhesion and signaling","volume":"21","author":"Godsel","year":"2009","journal-title":"Curr. Opin. Cell Biol."},{"key":"ref_84","doi-asserted-by":"crossref","unstructured":"Puzzi, L., Borin, D., Gurha, P., Lombardi, R., Martinelli, V., Weiss, M., Andolfi, L., Lazzarino, M., Mestroni, L., and Marian, A.J. (2019). Knock Down of plakophillin 2 dysregulates adhesion pathway through upregulation of miR200b and alters the mechanical properties in cardiac cells. Cells, 8.","DOI":"10.3390\/cells8121639"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"dev146589","DOI":"10.1242\/dev.146589","article-title":"Wnt signaling in development and tissue homeostasis","volume":"145","author":"Steinhart","year":"2018","journal-title":"Development"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1248012","DOI":"10.1126\/science.1248012","article-title":"Stem cell signaling. An integral program for tissue renewal and regeneration: Wnt signaling and stem cell control","volume":"346","author":"Clevers","year":"2014","journal-title":"Science"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"68","DOI":"10.4161\/org.4.2.5851","article-title":"Wnt signal transduction pathways","volume":"4","author":"Komiya","year":"2008","journal-title":"Organogenesis"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"a007880","DOI":"10.1101\/cshperspect.a007880","article-title":"Frizzled and LRP5\/6 receptors for Wnt\/beta-catenin signaling","volume":"4","author":"MacDonald","year":"2012","journal-title":"Cold Spring Harb. Perspect Biol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1768","DOI":"10.1101\/gad.13.14.1768","article-title":"Wnt-induced dephosphorylation of axin releases beta-catenin from the axin complex","volume":"13","author":"Willert","year":"1999","journal-title":"Genes Dev."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.devcel.2009.06.016","article-title":"Wnt\/beta-catenin signaling: Components, mechanisms, and diseases","volume":"17","author":"MacDonald","year":"2009","journal-title":"Dev. Cell"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"950","DOI":"10.1126\/science.289.5481.950","article-title":"Inhibition of adipogenesis by Wnt signaling","volume":"289","author":"Ross","year":"2000","journal-title":"Science"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1083\/jcb.152.1.87","article-title":"Wnt-1 signaling inhibits apoptosis by activating beta-catenin\/T cell factor-mediated transcription","volume":"152","author":"Chen","year":"2001","journal-title":"J. Cell Biol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"38239","DOI":"10.1074\/jbc.M206402200","article-title":"MacDougald, O.A. Wnt signaling protects 3T3-L1 preadipocytes from apoptosis through induction of insulin-like growth factors","volume":"277","author":"Longo","year":"2002","journal-title":"J. Biol. Chem."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1016\/j.cell.2015.10.044","article-title":"Hippo Pathway in Organ Size Control, Tissue Homeostasis, and Cancer","volume":"163","author":"Yu","year":"2015","journal-title":"Cell"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1016\/j.molcel.2016.10.034","article-title":"Characterization of Hippo Pathway Components by Gene Inactivation","volume":"64","author":"Plouffe","year":"2016","journal-title":"Mol. Cell"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"642","DOI":"10.15252\/embr.201438638","article-title":"The Hippo signaling pathway in stem cell biology and cancer","volume":"15","author":"Mo","year":"2014","journal-title":"EMBO Rep."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"2123","DOI":"10.3892\/etm.2017.4246","article-title":"Transforming growth factor beta and its role in heart disease","volume":"13","author":"Liu","year":"2017","journal-title":"Exp. Ther. Med."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"a011197","DOI":"10.1101\/cshperspect.a011197","article-title":"Signaling by the TGFbeta superfamily","volume":"5","author":"Wrana","year":"2013","journal-title":"Cold Spring Harb. Perspect Biol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1002\/iub.239","article-title":"The tale of transforming growth factor-beta (TGFbeta) signaling: A soigne enigma","volume":"61","author":"Chaudhury","year":"2009","journal-title":"IUBMB Life"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"4582","DOI":"10.1093\/hmg\/ddr392","article-title":"Restrictive loss of plakoglobin in cardiomyocytes leads to arrhythmogenic cardiomyopathy","volume":"20","author":"Li","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/s40119-018-0104-3","article-title":"A Review of Interleukin-1 in Heart Disease: Where Do We Stand Today?","volume":"7","author":"Szekely","year":"2018","journal-title":"Cardiol. Ther."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"864","DOI":"10.1007\/s10495-010-0498-4","article-title":"IL-1beta induces ER stress in a JNK dependent manner that determines cell death in human pancreatic epithelial MIA PaCa-2 cells","volume":"15","author":"Verma","year":"2010","journal-title":"Apoptosis"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1016\/j.mcn.2007.07.005","article-title":"Interleukin-1beta mediates proliferation and differentiation of multipotent neural precursor cells through the activation of SAPK\/JNK pathway","volume":"36","author":"Wang","year":"2007","journal-title":"Mol. Cell Neurosci."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1146\/annurev.physiol.64.083101.145105","article-title":"Cardiac ion channels","volume":"64","author":"Roden","year":"2002","journal-title":"Annu. Rev. Physiol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1073\/pnas.0510519103","article-title":"A mutation in the human phospholamban gene, deleting arginine 14, results in lethal, hereditary cardiomyopathy","volume":"103","author":"Haghighi","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1161\/CIRCRESAHA.117.310230","article-title":"Calcium and Excitation-Contraction Coupling in the Heart","volume":"121","author":"Eisner","year":"2017","journal-title":"Circ. Res."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1021\/bi701668v","article-title":"Structural and dynamic basis of phospholamban and sarcolipin inhibition of Ca(2+)-ATPase","volume":"47","author":"Traaseth","year":"2008","journal-title":"Biochemistry"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.jbspin.2006.10.001","article-title":"Cardiovascular disease in rheumatoid arthritis: Single-center hospital-based cohort study in France","volume":"74","author":"Assous","year":"2007","journal-title":"Joint Bone Spine"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1007\/s12471-018-1211-4","article-title":"Prevalence and cardiac phenotype of patients with a phospholamban mutation","volume":"27","author":"Hof","year":"2019","journal-title":"Neth Heart J."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"9819","DOI":"10.1038\/s41598-020-66656-9","article-title":"The phospholamban p.(Arg14del) pathogenic variant leads to cardiomyopathy with heart failure and is unreponsive to standard heart failure therapy","volume":"10","author":"Eijgenraam","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/0167-5273(88)90212-4","article-title":"Juvenile sudden death and effort ventricular tachycardias in a family with right ventricular cardiomyopathy","volume":"21","author":"Nava","year":"1988","journal-title":"Int. J. Cardiol."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"12179","DOI":"10.1073\/pnas.0600268103","article-title":"Mice with the R176Q cardiac ryanodine receptor mutation exhibit catecholamine-induced ventricular tachycardia and cardiomyopathy","volume":"103","author":"Kannankeril","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/S0006-291X(02)02829-2","article-title":"Increased Ca2+ storage capacity in the sarcoplasmic reticulum by overexpression of HRC (histidine-rich Ca2+ binding protein)","volume":"300","author":"Kim","year":"2003","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1038\/nature11799","article-title":"Studying arrhythmogenic right ventricular dysplasia with patient-specific iPSCs","volume":"494","author":"Kim","year":"2013","journal-title":"Nature"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1038\/s41421-019-0095-9","article-title":"Single-cell imaging and transcriptomic analyses of endogenous cardiomyocyte dedifferentiation and cycling","volume":"5","author":"Zhang","year":"2019","journal-title":"Cell Discov."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/j.bbrc.2005.03.007","article-title":"Wnt5b partially inhibits canonical Wnt\/beta-catenin signaling pathway and promotes adipogenesis in 3T3-L1 preadipocytes","volume":"330","author":"Kanazawa","year":"2005","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1038\/nature07221","article-title":"New role of bone morphogenetic protein 7 in brown adipogenesis and energy expenditure","volume":"454","author":"Tseng","year":"2008","journal-title":"Nature"},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"3654","DOI":"10.1242\/jcs.086629","article-title":"Fibrosis and adipogenesis originate from a common mesenchymal progenitor in skeletal muscle","volume":"124 (Pt 21)","author":"Uezumi","year":"2011","journal-title":"J. Cell Sci."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1038\/ncb2014","article-title":"Mesenchymal progenitors distinct from satellite cells contribute to ectopic fat cell formation in skeletal muscle","volume":"12","author":"Uezumi","year":"2010","journal-title":"Nat. Cell Biol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1007\/s00018-007-6522-3","article-title":"Epicardium-derived cells in cardiogenesis and cardiac regeneration","volume":"64","author":"Winter","year":"2007","journal-title":"Cell Mol. Life Sci."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"1719","DOI":"10.1089\/scd.2014.0019","article-title":"Re-activated adult epicardial progenitor cells are a heterogeneous population molecularly distinct from their embryonic counterparts","volume":"23","author":"Bollini","year":"2014","journal-title":"Stem Cells Dev."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"640","DOI":"10.1038\/nature10188","article-title":"De novo cardiomyocytes from within the activated adult heart after injury","volume":"474","author":"Smart","year":"2011","journal-title":"Nature"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1161\/CIRCULATIONAHA.116.022064","article-title":"Insulin-Like Growth Factor 1 Receptor-Dependent Pathway Drives Epicardial Adipose Tissue Formation After Myocardial Injury","volume":"135","author":"Zangi","year":"2017","journal-title":"Circulation"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"1894","DOI":"10.1172\/JCI45529","article-title":"Adult mouse epicardium modulates myocardial injury by secreting paracrine factors","volume":"121","author":"Zhou","year":"2011","journal-title":"J. Clin. Invest."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1016\/j.celrep.2016.04.027","article-title":"Hippo Signaling Mediators Yap and Taz Are Required in the Epicardium for Coronary Vasculature Development","volume":"15","author":"Singh","year":"2016","journal-title":"Cell Rep."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"153.e6.","DOI":"10.1016\/j.devcel.2018.03.019","article-title":"Hippo Signaling Plays an Essential Role in Cell State Transitions during Cardiac Fibroblast Development","volume":"45","author":"Xiao","year":"2018","journal-title":"Dev. Cell"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1172\/JCI88759","article-title":"Epicardial YAP\/TAZ orchestrate an immunosuppressive response following myocardial infarction","volume":"127","author":"Ramjee","year":"2017","journal-title":"J. Clin. Invest."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"73","DOI":"10.3109\/15419061.2011.621561","article-title":"Plakophilin-2 and the migration, differentiation and transformation of cells derived from the epicardium of neonatal rat hearts","volume":"18","author":"Matthes","year":"2011","journal-title":"Cell Commun. Adhes"},{"key":"ref_129","unstructured":"Lombardi, R., Chen, S.N., Gurha, P., Czernuszewicz, G.Z., Willerson, J.T., and Marian, A.J. (2017). Desmoplakin Deficient Epicardial Progenitor Cells Mediate the Development of Fibroadiposis in Arrhythmogenic Cardiomyopathy. Circulation, 136, A20536."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1038\/cr.2014.125","article-title":"Epicardium-to-fat transition in injured heart","volume":"24","author":"Liu","year":"2014","journal-title":"Cell Res."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"2070","DOI":"10.1073\/pnas.1417232112","article-title":"Adipogenesis and epicardial adipose tissue: A novel fate of the epicardium induced by mesenchymal transformation and PPARgamma activation","volume":"112","author":"Yamaguchi","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"1787","DOI":"10.1084\/jem.20090641","article-title":"Myocyte necrosis underlies progressive myocardial dystrophy in mouse dsg2-related arrhythmogenic right ventricular cardiomyopathy","volume":"206","author":"Pilichou","year":"2009","journal-title":"J. Exp. Med."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"2079","DOI":"10.1007\/s00259-010-1525-y","article-title":"Assessment of inflammation in patients with arrhythmogenic right ventricular cardiomyopathy\/dysplasia","volume":"37","author":"Campian","year":"2010","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1016\/S0140-6736(94)92339-6","article-title":"Evidence from family studies for autoimmunity in dilated cardiomyopathy","volume":"344","author":"Caforio","year":"1994","journal-title":"Lancet"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1016\/S0092-8674(03)00687-1","article-title":"Adult cardiac stem cells are multipotent and support myocardial regeneration","volume":"114","author":"Beltrami","year":"2003","journal-title":"Cell"},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1161\/CIRCRESAHA.115.305373","article-title":"Emerging Concepts in Paracrine Mechanisms in Regenerative Cardiovascular Medicine and Biology","volume":"118","author":"Hodgkinson","year":"2016","journal-title":"Circ. Res."},{"key":"ref_137","doi-asserted-by":"crossref","unstructured":"Mancuso, T., Barone, A., Salatino, A., Molinaro, C., Marino, F., Scalise, M., Torella, M., Angelis, A.D., Urbanek, K., and Torella, D. (2020). Unravelling the Biology of Adult Cardiac Stem Cell-Derived Exosomes to Foster Endogenous Cardiac Regeneration and Repair. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21103725"},{"key":"ref_138","doi-asserted-by":"crossref","unstructured":"Routledge, D., and Scholpp, S. (2019). Mechanisms of intercellular Wnt transport. Development, 146.","DOI":"10.1242\/dev.176073"},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1146\/annurev-biochem-013118-111902","article-title":"Exosomes","volume":"88","author":"Pegtel","year":"2019","journal-title":"Annu. Rev. Biochem."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"106","DOI":"10.4252\/wjsc.v10.i8.106","article-title":"Stem cell-derived exosomes\u2014An emerging tool for myocardial regeneration","volume":"10","author":"Lazar","year":"2018","journal-title":"World J. Stem Cells"},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1016\/j.hrthm.2016.12.010","article-title":"Use of flecainide in combination antiarrhythmic therapy in patients with arrhythmogenic right ventricular cardiomyopathy","volume":"14","author":"Ermakov","year":"2017","journal-title":"Heart Rhythm."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/21\/17\/6320\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:05:12Z","timestamp":1760177112000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/21\/17\/6320"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,31]]},"references-count":141,"journal-issue":{"issue":"17","published-online":{"date-parts":[[2020,9]]}},"alternative-id":["ijms21176320"],"URL":"https:\/\/doi.org\/10.3390\/ijms21176320","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,8,31]]}}}