Given the role of HSPB5 protein in the remodeling of the cytoskeleton during development and cell differentiation, as well as stress conditions, it is not surprising that the myofibrillar myopathy (MFM) of skeletal and cardiac muscle, including desmin-related myopathies (DRM) and dilated (DCM) and restrictive (RCM) cardiomyopathy [21,23,30,111], are caused by mutations of this sHSP gene (Table 3). This evidence concerns the gene DES and familial dilated cardiomyopathy.