In summary, our data add a further insight in the pathogenesis of LMNA-RM, demonstrating that LMNA-RM muscle tissue is characterized by the over-expression of TLR7 and TLR9, probably due to the release of danger signals, as supposed for DMD, or to a NF-κB signaling impairment. Here, TLR9 is linked to Duchenne muscular dystrophy.