Our data showed that enhanced ERK activity is also observed in heart tissue from the nesprin-1 KASH KO mice, fibroblasts derived from EDMD-DCM patients and nesprin-1 mutant transfected cells, suggesting that nesprin and lamin A/C function through a similar pathway and trigger up-regulation of ERK. This evidence concerns the gene SYNE1 and Emery-Dreifuss muscular dystrophy.