Established and Emerging Mechanisms in the Pathogenesis of Arrhythmogenic Cardiomyopathy: A Multifaceted Disease

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Exosomes

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Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Proposed Modification of the Task Force Criteria

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Inhibition of Adipogenesis by Wnt Signaling

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Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: Proposed Modification of the Task Force Criteria

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Mesenchymal progenitors distinct from satellite cells contribute to ectopic fat cell formation in skeletal muscle

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Identification of a deletion in plakoglobin in arrhythmogenic right ventricular cardiomyopathy with palmoplantar keratoderma and woolly hair (Naxos disease)

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Arrhythmogenic Right Ventricular Cardiomyopathy: Dysplasia, Dystrophy, or Myocarditis?

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Calcium and Excitation-Contraction Coupling in the Heart

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Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy

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Wnt signaling in development and tissue homeostasis

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Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy, woolly hair and keratoderma

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Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2)

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The Hippo signaling pathway in stem cell biology and cancer

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Fibrosis and adipogenesis originate from a common mesenchymal progenitor in skeletal muscle

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Mutation in Human Desmoplakin Domain Binding to Plakoglobin Causes a Dominant Form of Arrhythmogenic Right Ventricular Cardiomyopathy

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Left-Dominant Arrhythmogenic Cardiomyopathy

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De novo cardiomyocytes from within the activated adult heart after injury

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Suppression of canonical Wnt/ -catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy

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Frizzled and LRP5/6 Receptors for Wnt/ -Catenin SignalingPreprint

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Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria

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Mutations in Desmoglein-2 Gene Are Associated With Arrhythmogenic Right Ventricular Cardiomyopathy

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Clinical and Genetic Characterization of Families With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Provides Novel Insights Into Patterns of Disease Expression

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Declining Risk of Sudden Death in Heart Failure

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Arrhythmogenic Right Ventricular Cardiomyopathy Type 5 Is a Fully Penetrant, Lethal Arrhythmic Disorder Caused by a Missense Mutation in the TMEM43 Gene

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Adult mouse epicardium modulates myocardial injury by secreting paracrine factors

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Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Patients and Family Members

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Studying arrhythmogenic right ventricular dysplasia with patient-specific iPSCs

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Truncating FLNC Mutations Are Associated With High-Risk Dilated and Arrhythmogenic Cardiomyopathies

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Phospholamban R14del Mutation in Patients Diagnosed with Dilated Cardiomyopathy or Arrhythmogenic Right Ventricular Cardiomyopathy: Evidence Supporting the Concept of Arrhythmogenic Cardiomyopathy

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Regulatory mutations in transforming growth factor-?3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1

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WNT-1 Signaling Inhibits Apoptosis by Activating β-Catenin/T Cell Factor–Mediated Transcription

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A mutation in the human phospholamban gene, deleting arginine 14, results in lethal, hereditary cardiomyopathy

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Plakophilin-2 Mutations Are the Major Determinant of Familial Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

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Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease)

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Wnt-induced dephosphorylation of Axin releases beta -catenin from the Axin complexPreprint

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Missense Mutations in Plakophilin-2 Cause Sodium Current Deficit and Associate With a Brugada Syndrome Phenotype

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Loss of Plakophilin-2 Expression Leads to Decreased Sodium Current and Slower Conduction Velocity in Cultured Cardiac Myocytes

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Cardiac Ion Channels

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The Cardiac Desmosome and Arrhythmogenic Cardiomyopathies

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Genetic Variation in Titin in Arrhythmogenic Right Ventricular Cardiomyopathy–Overlap Syndromes

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Emerging Concepts in Paracrine Mechanisms in Regenerative Cardiovascular Medicine and Biology

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The Hippo Pathway Is Activated and Is a Causal Mechanism for Adipogenesis in Arrhythmogenic Cardiomyopathy

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Characterization of Hippo Pathway Components by Gene Inactivation

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Mutant Desmocollin-2 Causes Arrhythmogenic Right Ventricular Cardiomyopathy

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Pathophysiology of arrhythmogenic cardiomyopathy

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Mutations in the Lamin A/C gene mimic arrhythmogenic right ventricular cardiomyopathy

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A Novel Dominant Mutation in Plakoglobin Causes Arrhythmogenic Right Ventricular Cardiomyopathy

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Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy: spectrum of mutations and clinical impact in practice

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Connexin43 Remodeling Caused by Inhibition of Plakophilin-2 Expression in Cardiac Cells

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Wnt Signaling Protects 3T3-L1 Preadipocytes from Apoptosis through Induction of Insulin-like Growth Factors

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Intercalated disc abnormalities, reduced Na+ current density, and conduction slowing in desmoglein-2 mutant mice prior to cardiomyopathic changes

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Plakophilin-2 Is Required for Transcription of Genes That Control Calcium Cycling and Cardiac Rhythm

Cerrone, M., Montnach, J., Lin, X., Zhao, Y.-T., Zhang, M., Agullo-Pascual, E., Leo-Macias, A., Alvarado, F. J., Dolgalev, I., Karathanos, T. V., et al. (2017) · Nature Communications · doi:10.1038/s41467-017-00127-0 · Cited by 210 · see citing works

Comprehensive Desmosome Mutation Analysis in North Americans With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

den Haan, A. D., Tan, B. Y., Zikusoka, M. N., Ibáñez, L., Jain, R., Daly, A., Tichnell, C., James, C. A., Amat‐Alarcon, N., Abraham, T. P., et al. (2009) · Circulation Cardiovascular Genetics · doi:10.1161/circgenetics.109.858217 · Cited by 210 · see citing works

Myocyte necrosis underlies progressive myocardial dystrophy in mouse <i>dsg2</i> -related arrhythmogenic right ventricular cardiomyopathy

Pilichou, K., Remme, C. A., Basso, C., Campian, M. E., Rizzo, S., Barnett, P., Scicluna, B. P., Bauce, B., van den Hoff, M. J. B., de Bakker, J. M. T., et al. (2009) · Journal of Experimental Medicine · doi:10.1084/jem.20090641 · Cited by 210 · see citing works

Mutations in the area composita protein αT-catenin are associated with arrhythmogenic right ventricular cardiomyopathy

van Hengel, J., Calore, M., Bauce, B., Dazzo, E., Mazzotti, E., Bortoli, M. D., Lorenzon, A., Mura, I. E. A. L., Beffagna, G., Rigato, I., et al. (2012) · European Heart Journal · doi:10.1093/eurheartj/ehs373 · Cited by 196 · see citing works

2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: Executive summary

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Therapeutic Modulation of the Immune Response in Arrhythmogenic Cardiomyopathy

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Mice with the R176Q cardiac ryanodine receptor mutation exhibit catecholamine-induced ventricular tachycardia and cardiomyopathy

Kannankeril, P. J., Mitchell, B. M., Goonasekera, S. A., Chelu, M. G., Zhang, W., Sood, S., Kearney, D. L., Danila, C. I., Biasi, M. D., Wehrens, X. H. T., et al. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0600268103 · Cited by 190 · see citing works

De novo desmin-mutation N116S is associated with arrhythmogenic right ventricular cardiomyopathy

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Epicardial YAP/TAZ orchestrate an immunosuppressive response following myocardial infarction

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Hippo Signaling Plays an Essential Role in Cell State Transitions during Cardiac Fibroblast Development

Xiao, Y., Hill, M. C., Zhang, M., Martin, T. J., Morikawa, Y., Wang, S., Moise, A. R., Wythe, J. D., & Martin, J. F. (2018) · Developmental Cell · doi:10.1016/j.devcel.2018.03.019 · Cited by 183 · see citing works

Adipogenesis and epicardial adipose tissue: A novel fate of the epicardium induced by mesenchymal transformation and PPARγ activation

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Multilevel analyses of SCN5A mutations in arrhythmogenic right ventricular dysplasia/cardiomyopathy suggest non-canonical mechanisms for disease pathogenesis

Riele, A. S. J. M. T., Agulló-Pascual, E., James, C. A., Leo‐Macías, A., Cerrone, M., Zhang, M., Lin, X., Lin, B., Rothenberg, E., Sobreira, N. L. D. M., et al. (2016) · Cardiovascular Research · doi:10.1093/cvr/cvw234 · Cited by 169 · see citing works

Severe cardiac phenotype with right ventricular predominance in a large cohort of patients with a single missense mutation in the DES gene

van Tintelen, J. P., Gelder, I. C. V., Asimaki, A., Suurmeijer, A. J. H., Wiesfeld, A. C. P., Jongbloed, J. D. H., van den Wijngaard, A., Kuks, J. B. M., van Spaendonck‐Zwarts, K. Y., Notermans, N. C., et al. (2009) · Heart Rhythm · doi:10.1016/j.hrthm.2009.07.041 · Cited by 168 · see citing works

Evidence from family studies for autoimmunity in dilated cardiomyopathy

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Plakophilins: multifunctional scaffolds for adhesion and signaling

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