Two further novel desmin mutations—p.N116S, which is located in segment 1A of the desmin rod domain [161], and p.Glu401Asp, which is located in segment Coil 2B [162]—have recently been reported associated with inherited left ventricular arrhythmogenic cardiomyopathy/dysplasia, with a high incidence of adverse clinical events in the absence of skeletal myopathy or conduction system disorders. This evidence concerns the gene DES and Skeletal myopathy.