Clinically relevant mouse models of cardiac proteotoxicity, such as the mutant desmin transgenic mouse with 7-amino acid deletion R172-E178 in desmin (D7-Des Tg) (Goldfarb et al., 1998; Dalakas et al., 2000; Wang et al., 2001; Zheng et al., 2011), display a collapse of the desmin network and an accumulation of desmin aggregates that contributes to the development of cardiomyopathy (Wang et al., 2001). Here, DES is linked to cardiomyopathy.