The allele frequency of the DSG2 p.Arg119Ter variant among 808 patients with nonischemic cardiomyopathy, including one homozygous patient and four heterozygous patients, was 0.0037, which was more than 50-fold greater than that reported in the general Japanese population (0.0000064)18. Here, DSG2 is linked to nonischemic cardiomyopathy.