We previously have reported on the pathogenic effects of the human R406W desmin missense mutation in a young male patient with progressive restrictive cardiomyopathy, cardiac conduction defects and arrhythmias necessitating heart transplantation as well as on the generation and characterization of hetero‐ and homozygous R405W desmin knock‐in mice, which harbour the ortholog of the human R406W desmin mutation [17]. This evidence concerns the gene DES and restrictive cardiomyopathy.