Consistent with the important biological functions of the LINC complex in mechanical stress response, mutations in different components of the LINC complex and its interaction partners (Nesprin-1 and Nesprin-2, lamin A, Emerin, and TMEM43) are associated with diseases of the heart and striated muscles that experience high levels of mechanical stress, including dilated cardiomyopathy (DCM), arrhythmogenic cardiomyopathy (ACM), and Emery-Dreifuss muscular dystrophy (EDMD) (Stroud et al., 2014). Here, TMEM43 is linked to Emery-Dreifuss muscular dystrophy.