Published in Circulation on February 27, 2006
Multidisciplinary Study of Right Ventricular Dysplasia | NCT00024505
Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation (2010) 6.71
Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J (2010) 4.13
Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy. J Clin Invest (2006) 3.22
Mechanisms of disease: molecular genetics of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Nat Clin Pract Cardiovasc Med (2008) 3.19
Arrhythmogenic right ventricular cardiomyopathy type 5 is a fully penetrant, lethal arrhythmic disorder caused by a missense mutation in the TMEM43 gene. Am J Hum Genet (2008) 2.84
Arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in the desmosomal gene desmocollin-2. Am J Hum Genet (2006) 2.26
Comprehensive desmosome mutation analysis in north americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Cardiovasc Genet (2009) 2.19
Clinical phenotype and diagnosis of arrhythmogenic right ventricular cardiomyopathy in pediatric patients carrying desmosomal gene mutations. Heart Rhythm (2011) 2.11
Loss of mXinalpha, an intercalated disk protein, results in cardiac hypertrophy and cardiomyopathy with conduction defects. Am J Physiol Heart Circ Physiol (2007) 1.99
A novel dominant mutation in plakoglobin causes arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet (2007) 1.91
Remodeling of the cardiac sodium channel, connexin43, and plakoglobin at the intercalated disk in patients with arrhythmogenic cardiomyopathy. Heart Rhythm (2012) 1.91
The desmosome. Cold Spring Harb Perspect Biol (2009) 1.77
Molecular composition of the intercalated disc in a spontaneous canine animal model of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Heart Rhythm (2007) 1.70
Mutant desmocollin-2 causes arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet (2006) 1.68
Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Orphanet J Rare Dis (2007) 1.64
Compound and digenic heterozygosity contributes to arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol (2010) 1.62
Incremental value of cardiac magnetic resonance imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol (2013) 1.58
The desmosome and pemphigus. Histochem Cell Biol (2008) 1.54
Nuclear plakoglobin is essential for differentiation of cardiac progenitor cells to adipocytes in arrhythmogenic right ventricular cardiomyopathy. Circ Res (2011) 1.47
Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia. J Cardiovasc Magn Reson (2008) 1.45
Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a review and update. Clin Res Cardiol (2011) 1.37
Cell-cell connection to cardiac disease. Trends Cardiovasc Med (2009) 1.34
Desmoglein as a target in skin disease and beyond. J Invest Dermatol (2011) 1.28
Pathophysiology of arrhythmogenic cardiomyopathy. Nat Rev Cardiol (2011) 1.25
Arrhythmogenic right ventricular cardiomyopathy/dysplasia on the basis of the revised diagnostic criteria in affected families with desmosomal mutations. Eur Heart J (2011) 1.24
Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare "disease of the desmosome" with multiple clinical presentations. Clin Res Cardiol (2009) 1.20
Mutations in genes encoding the cadherin receptor-ligand pair DCHS1 and FAT4 disrupt cerebral cortical development. Nat Genet (2013) 1.18
Abnormal connexin43 in arrhythmogenic right ventricular cardiomyopathy caused by plakophilin-2 mutations. J Cell Mol Med (2008) 1.18
Myocyte necrosis underlies progressive myocardial dystrophy in mouse dsg2-related arrhythmogenic right ventricular cardiomyopathy. J Exp Med (2009) 1.18
Mechanistic basis of desmosome-targeted diseases. J Mol Biol (2013) 1.16
Desmosomes at a glance. J Cell Sci (2009) 1.14
Recessive arrhythmogenic right ventricular dysplasia due to novel cryptic splice mutation in PKP2. Hum Mutat (2006) 1.13
The biology of the desmosome-like junction a versatile anchoring junction and signal transducer in the seminiferous epithelium. Int Rev Cell Mol Biol (2011) 1.12
Genetics of inherited cardiomyopathy. Eur Heart J (2011) 1.11
Missense mutations in desmocollin-2 N-terminus, associated with arrhythmogenic right ventricular cardiomyopathy, affect intracellular localization of desmocollin-2 in vitro. BMC Med Genet (2007) 1.11
Autoimmune and infectious skin diseases that target desmogleins. Proc Jpn Acad Ser B Phys Biol Sci (2010) 1.03
Exercise has a disproportionate role in the pathogenesis of arrhythmogenic right ventricular dysplasia/cardiomyopathy in patients without desmosomal mutations. J Am Heart Assoc (2014) 1.03
The junctions that don't fit the scheme: special symmetrical cell-cell junctions of their own kind. Cell Tissue Res (2009) 1.03
Arrhythmogenic right ventricular dysplasia, a cell adhesion cardiomyopathy: insights into disease pathogenesis from preliminary genotype--phenotype assessment. Heart (2006) 1.02
Molecular genetics and pathogenesis of cardiomyopathy. J Hum Genet (2015) 1.02
Mechanotransduction in cardiac hypertrophy and failure. Circ Res (2015) 1.01
A new human DSG2-transgenic mouse model for studying the tropism and pathology of human adenoviruses. J Virol (2012) 1.00
Arrhythmogenic right ventricular cardiomyopathy: moving toward mechanism. J Clin Invest (2006) 0.99
Broken hearts, woolly hair, and tattered skin: when desmosomal adhesion goes awry. Curr Opin Cell Biol (2007) 0.99
Peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension. Circulation (2014) 0.98
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review. Clin Med Insights Cardiol (2013) 0.97
Molecular genetics and pathogenesis of arrhythmogenic right ventricular cardiomyopathy: a disease of cardiac stem cells. Pediatr Cardiol (2011) 0.92
Arrhythmogenic right ventricular cardiomyopathy (ARVC): cardiovascular magnetic resonance update. J Cardiovasc Magn Reson (2014) 0.92
Arrhythmogenic cardiomyopathy: diagnosis, genetic background, and risk management. Neth Heart J (2014) 0.91
Recurrent and founder mutations in the Netherlands : Plakophilin-2 p.Arg79X mutation causing arrhythmogenic right ventricular cardiomyopathy/dysplasia. Neth Heart J (2010) 0.91
Arrhythmogenic right ventricular cardiomyopathy is a disease of cardiac stem cells. Curr Opin Cardiol (2010) 0.90
The p.A897KfsX4 frameshift variation in desmocollin-2 is not a causative mutation in arrhythmogenic right ventricular cardiomyopathy. Eur J Hum Genet (2010) 0.89
Differential expression of the cardiac ryanodine receptor in normal and arrhythmogenic right ventricular cardiomyopathy canine hearts. Hum Genet (2006) 0.89
Defects in cytoskeletal signaling pathways, arrhythmia, and sudden cardiac death. Front Physiol (2012) 0.89
Single molecule force spectroscopy on titin implicates immunoglobulin domain stability as a cardiac disease mechanism. J Biol Chem (2013) 0.88
Mutations with pathogenic potential in proteins located in or at the composite junctions of the intercalated disk connecting mammalian cardiomyocytes: a reference thesaurus for arrhythmogenic cardiomyopathies and for Naxos and Carvajal diseases. Cell Tissue Res (2012) 0.88
Characterization of desmoglein expression in the normal prostatic gland. Desmoglein 2 is an independent prognostic factor for aggressive prostate cancer. PLoS One (2014) 0.88
Cardiac mesenchymal stromal cells are a source of adipocytes in arrhythmogenic cardiomyopathy. Eur Heart J (2015) 0.87
Novel missense mutations in exon 15 of desmoglein-2: role of the intracellular cadherin segment in arrhythmogenic right ventricular cardiomyopathy? Heart Rhythm (2010) 0.87
Connexin defects underlie arrhythmogenic right ventricular cardiomyopathy in a novel mouse model. Hum Mol Genet (2013) 0.87
Desmosomal molecules in and out of adhering junctions: normal and diseased States of epidermal, cardiac and mesenchymally derived cells. Dermatol Res Pract (2010) 0.86
Finding cardiovascular disease genes in the dog. J Vet Cardiol (2006) 0.86
Evolving molecular diagnostics for familial cardiomyopathies: at the heart of it all. Expert Rev Mol Diagn (2010) 0.85
Cadherins as targets for genetic diseases. Cold Spring Harb Perspect Biol (2010) 0.85
Cell-cell junction remodeling in the heart: possible role in cardiac conduction system function and arrhythmias? Life Sci (2011) 0.85
Noninvasive Multimodality Imaging in ARVD/C. JACC Cardiovasc Imaging (2015) 0.84
Mechanotransduction Mechanisms for Intraventricular Diastolic Vortex Forces and Myocardial Deformations: Part 2. J Cardiovasc Transl Res (2015) 0.84
Desmin mutations and arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol (2012) 0.84
Diagnostic dilemmas: overlapping features of brugada syndrome and arrhythmogenic right ventricular cardiomyopathy. Front Physiol (2012) 0.84
Prevalence and pathophysiologic attributes of ventricular dyssynchrony in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol (2009) 0.84
The electrocardiographic manifestations of arrhythmogenic right ventricular dysplasia. Curr Cardiol Rev (2014) 0.83
Gene Mutations Resulting in the Development of ARVC/D Could Affect Cells of the Cardiac Conduction System. Front Physiol (2012) 0.83
TMEM43 mutation p.S358L alters intercalated disc protein expression and reduces conduction velocity in arrhythmogenic right ventricular cardiomyopathy. PLoS One (2014) 0.83
Brugada ECG pattern: a physiopathological prospective study based on clinical, electrophysiological, angiographic, and genetic findings. Front Physiol (2012) 0.82
Markedly increased intracellular lipid droplets and disruption of intercellular junctions in biopsied myocardium from a patient with arrhythmogenic right ventricular cardiomyopathy. Heart Vessels (2008) 0.82
Passive ventricular remodeling in cardiac disease: focus on heterogeneity. Front Physiol (2014) 0.82
Identification of a PKP2 gene deletion in a family with arrhythmogenic right ventricular cardiomyopathy. Eur J Hum Genet (2013) 0.81
Alterations in cell adhesion proteins and cardiomyopathy. World J Cardiol (2014) 0.80
Mouse models in arrhythmogenic right ventricular cardiomyopathy. Front Physiol (2012) 0.80
The carboxy terminal domain of connexin43: from molecular regulation of the gap junction channel to supramolecular organization of the intercalated disk. Circ Res (2007) 0.80
Restricting sports for athletes with heart disease: are we saving lives, avoiding lawsuits, or just promoting obesity and sedentary living? Pediatr Cardiol (2012) 0.80
Genetics and Genomics of Single-Gene Cardiovascular Diseases: Common Hereditary Cardiomyopathies as Prototypes of Single-Gene Disorders. J Am Coll Cardiol (2016) 0.79
Desmosomal cadherins are decreased in explanted arrhythmogenic right ventricular dysplasia/cardiomyopathy patient hearts. PLoS One (2013) 0.78
Genetic factors in arrhythmogenic right ventricular cardiomyopathy: need for a DNA bank! Neth Heart J (2006) 0.78
Quantitative Immunohistochemistry of Desmosomal Proteins (Plakoglobin, Desmoplakin and Plakophilin), Connexin-43, and N-cadherin in Arrhythmogenic Cardiomyopathy: An Autopsy Study. Open Cardiovasc Med J (2013) 0.78
In vitro functional analyses of arrhythmogenic right ventricular cardiomyopathy-associated desmoglein-2-missense variations. PLoS One (2012) 0.77
Arrhythmogenic right ventricular cardiomyopathy: asymptomatic to life threatening as illustrated by the cases of two sisters. Neth Heart J (2007) 0.77
Do cell junction protein mutations cause an airway phenotype in mice or humans? Am J Respir Cell Mol Biol (2011) 0.77
Impact of new electrocardiographic criteria in arrhythmogenic cardiomyopathy. Front Physiol (2012) 0.77
Arrhythmogenic right ventricular dysplasia/cardiomyopathy type 1: a light on molecular mechanisms. Genet Res Int (2013) 0.77
Desmoglein-2 is overexpressed in non-small cell lung cancer tissues and its knockdown suppresses NSCLC growth by regulation of p27 and CDK2. J Cancer Res Clin Oncol (2016) 0.76
Geographical distribution of plakophilin-2 mutation prevalence in patients with arrhythmogenic cardiomyopathy. Neth Heart J (2012) 0.76
A Proteomics Approach to Identify New Putative Cardiac Intercalated Disk Proteins. PLoS One (2016) 0.76
Imaging phenotype vs genotype in nonhypertrophic heritable cardiomyopathies: dilated cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. Circ Cardiovasc Imaging (2010) 0.76
Rare Variants in Cardiomyopathy Genes Associated With Stress-Induced Cardiomyopathy. Neurosurgery (2016) 0.75
The genetic background of arrhythmogenic right ventricular cardiomyopathy. J Arrhythm (2016) 0.75
A global perspective of arrhythmogenic right ventricular cardiomyopathy. Glob Cardiol Sci Pract (2013) 0.75
Arrhythmogenic cardiomyopathy. Orphanet J Rare Dis (2016) 0.75
c-Src/Cav1-dependent activation of the EGFR by Dsg2. Oncotarget (2016) 0.75
Arrhythmogenic Right Ventricular Dysplasia in Neuromuscular Disorders. Clin Med Insights Cardiol (2016) 0.75
Guidelines on the diagnosis and management of pericardial diseases executive summary; The Task force on the diagnosis and management of pericardial diseases of the European society of cardiology. Eur Heart J (2004) 7.84
Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation (2010) 6.71
Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. JAMA (2006) 5.79
A new diagnostic test for arrhythmogenic right ventricular cardiomyopathy. N Engl J Med (2009) 4.54
Arrhythmogenic right ventricular cardiomyopathy. Lancet (2009) 4.46
Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J (2010) 4.13
Regulatory mutations in transforming growth factor-beta3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1. Cardiovasc Res (2005) 3.99
Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease). Heart Rhythm (2004) 3.54
Does sports activity enhance the risk of sudden death in adolescents and young adults? J Am Coll Cardiol (2003) 3.52
Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J (2013) 3.50
Guidelines on prevention, diagnosis and treatment of infective endocarditis executive summary; the task force on infective endocarditis of the European society of cardiology. Eur Heart J (2004) 3.45
Outcomes in athletes with marked ECG repolarization abnormalities. N Engl J Med (2008) 3.30
IDEG6: a web tool for detection of differentially expressed genes in multiple tag sampling experiments. Physiol Genomics (2003) 3.26
Desmosomal dysfunction due to mutations in desmoplakin causes arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Res (2006) 2.95
Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet (2002) 2.95
Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: results from the North American Multidisciplinary Study. Heart Rhythm (2009) 2.82
A prospective study of biopsy-proven myocarditis: prognostic relevance of clinical and aetiopathogenetic features at diagnosis. Eur Heart J (2007) 2.76
The International Society of Heart and Lung Transplantation Guidelines for the care of heart transplant recipients. J Heart Lung Transplant (2010) 2.59
Clinical profile of four families with arrhythmogenic right ventricular cardiomyopathy caused by dominant desmoplakin mutations. Eur Heart J (2005) 2.56
Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation (2003) 2.56
The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Circulation (2007) 2.50
Recommendations for interpretation of 12-lead electrocardiogram in the athlete. Eur Heart J (2009) 2.37
Novel definition files for human GeneChips based on GeneAnnot. BMC Bioinformatics (2007) 2.32
Myocardial edema underlies dynamic T-wave inversion (Wellens' ECG pattern) in patients with reversible left ventricular dysfunction. Heart Rhythm (2011) 2.18
Immunosuppressive therapy for active lymphocytic myocarditis: virological and immunologic profile of responders versus nonresponders. Circulation (2003) 2.12
Clinical phenotype and diagnosis of arrhythmogenic right ventricular cardiomyopathy in pediatric patients carrying desmosomal gene mutations. Heart Rhythm (2011) 2.11
Concealed metastatic lung carcinoma presenting as acute coronary syndrome with progressive conduction abnormalities. Circulation (2012) 2.10
Pre-participation screening of young competitive athletes for prevention of sudden cardiac death. J Am Coll Cardiol (2008) 2.09
Three-dimensional electroanatomical voltage mapping and histologic evaluation of myocardial substrate in right ventricular outflow tract tachycardia. J Am Coll Cardiol (2008) 2.06
Quantitative assessment of endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy/dysplasia: an in vitro validation of diagnostic criteria. Eur Heart J (2008) 2.05
Multiple mutations in desmosomal proteins encoding genes in arrhythmogenic right ventricular cardiomyopathy/dysplasia. Heart Rhythm (2009) 2.00
Aortic elasticity and size in bicuspid aortic valve syndrome. Eur Heart J (2007) 1.95
Long-term follow-up of individuals with the electrocardiographic pattern of right bundle-branch block and ST-segment elevation in precordial leads V1 to V3. Circulation (2002) 1.91
Identification and functional analysis of ZIC3 mutations in heterotaxy and related congenital heart defects. Am J Hum Genet (2003) 1.90
Structural and molecular pathology of the heart in Carvajal syndrome. Cardiovasc Pathol (2004) 1.82
Compound and digenic heterozygosity predicts lifetime arrhythmic outcome and sudden cardiac death in desmosomal gene-related arrhythmogenic right ventricular cardiomyopathy. Circ Cardiovasc Genet (2013) 1.81
Guidelines for autopsy investigation of sudden cardiac death. Virchows Arch (2007) 1.81
Analysis of the human VPS13 gene family. Genomics (2004) 1.65
Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Orphanet J Rare Dis (2007) 1.64
Compound and digenic heterozygosity contributes to arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol (2010) 1.62