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Cardiac cell-cell junctions in health and disease: Electrical versus mechanical coupling.
- M. Noorman, M. V. D. van der Heyden, +4 authors H. V. van Rijen
- Materials Science, Medicine
- Journal of molecular and cellular cardiology
- 1 July 2009
Intercalated discs are the membrane sites where individual cardiomyocytes are connected to each other. Adherens-, desmosomal-, and gap junctions are situated in the intercalated disc and ensure… Expand
Sodium current deficit and arrhythmogenesis in a murine model of plakophilin-2 haploinsufficiency.
- M. Cerrone, M. Noorman, +9 authors M. Delmar
- Biology, Medicine
- Cardiovascular research
- 1 September 2012
AIMS The shRNA-mediated loss of expression of the desmosomal protein plakophilin-2 leads to sodium current (I(Na)) dysfunction. Whether pkp2 gene haploinsufficiency leads to I(Na) deficit in vivo… Expand
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Pathogenic Desmosome Mutations in Index-Patients Predict Outcome of Family Screening Dutch Arrhythmogenic Right Ventricular…
Background— Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an autosomal dominant inherited disease with incomplete penetrance and variable expression. Causative mutations in… Expand
Dominant arrhythmia vulnerability of the right ventricle in senescent mice.
BACKGROUND Several cardiac disorders affect the right ventricle (RV) and left ventricle (LV) equally, but nevertheless, RV vulnerability to conduction slowing and arrhythmias exceeds that of the LV.… Expand
Reduced heterogeneous expression of Cx43 results in decreased Nav1.5 expression and reduced sodium current that accounts for arrhythmia vulnerability in conditional Cx43 knockout mice.
BACKGROUND Reduced expression of connexin43 (Cx43) and sodium channel (Nav1.5) and increased expression of collagen (fibrosis) are important determinants of impulse conduction in the heart. … Expand
Remodeling of the cardiac sodium channel, connexin43, and plakoglobin at the intercalated disk in patients with arrhythmogenic cardiomyopathy.
BACKGROUND Arrhythmogenic cardiomyopathy (AC) is closely associated with desmosomal mutations in a majority of patients. Arrhythmogenesis in patients with AC is likely related to remodeling of… Expand
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Diagnostic Task Force Criteria: Impact of New Task Force Criteria
- M. Cox, J. J. van der Smagt, +14 authors R. Hauer
- Circulation. Arrhythmia and electrophysiology
- 1 April 2010
Background—Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) Diagnostic Task Force Criteria (TFC) proposed in 1994 are highly specific but lack sensitivity. A new international task… Expand
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is a disease characterized by progressive fibrofatty replacement of primarily the right ventricle (RV). 1–3
Geographical distribution of plakophilin-2 mutation prevalence in patients with arrhythmogenic cardiomyopathy
- K. Jacob, M. Noorman, M. G. P. J. Cox, J. Groeneweg, R. Hauer, M. A. G. Heyden
- Netherlands Heart Journal
- 12 April 2012
Arrhythmogenic cardiomyopathy (AC) is characterised by myocardial fibrofatty tissue infiltration and presents with palpitations, ventricular arrhythmias, syncope and sudden cardiac death. AC is… Expand
Transmural dispersion of refractoriness and conduction velocity is associated with heterogeneously reduced connexin43 in a rabbit model of heart failure.
BACKGROUND Heterogeneity of repolarization and conduction is a potential source of arrhythmogenesis. In heart failure (HF), intercellular coupling is reduced and heterogeneities may become evident… Expand