Importantly, the cardiomyopathy observed in the Krusche et al. [43] mice, characterised by fibrotic lesions in the heart, was also evident in our strain of Dsg2lo/lo mice and is in keeping with the human clinical condition associated with DSG2 ‘loss-of-function’, arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) [5, 6]. This evidence concerns the gene DSG2 and arrhythmogenic right ventricular cardiomyopathy.