DES and Desminopathy: More than 70 mutations in the desmin gene have been associated with desminopathies (Capetanaki et al., 2015), and six of them (Ser12Phe, Ser13Phe, Arg16Cys, Ala285Val, Ser298Leu, Asp312Asn) are located within the NLSs on desmin (Szeverenyi et al., 2008) (Figure 2).