To investigate whether nesprin-1 defects lead to aberrant ERK1/2 activation, protein lysates from human dermal fibroblasts (HDFs) derived from EDMD and DCM patients carrying either nesprin-1, lamin A/C or emerin mutations (13), and heart tissue from WT and nesprin-1 KASH KO mice with EDMD-like phenotype and DCM (21) were examined. Here, EMD is linked to familial dilated cardiomyopathy.