Arrhythmogenic ventricular cardiomyopathy: A paradigm shift from right to biventricular disease

Figure 2 Molecular model of the desmosome: in the desmosomal complex the intermediate filaments of the cytoskeleton (desmin in the heart) are linked to the transmembranous cadherins (desmocollin and desmoglein) via armadillo proteins (plakoglobin and plakophilin) and desmoplakin. This interaction is crucial for myocardial mechanical and electrical stability. Mutations in arrhythmogenic right ventricular cardiomyopathy mostly affect desmosomal proteins.