In keeping with its role in tissue integrity, loss of DSG2 function in humans is directly linked to arrhythmogenic right ventricular cardiomyopathy (ARVC), an autosomal recessive disease underpinned by myocyte apoptosis and fibrous degeneration of the myocardium [5, 6]. Here, DSG2 is linked to Arrhythmogenic right ventricular dysplasia.