LMNA and laminopathy: Two major mouse models of laminopathy, lamin A/C-null (Lmna-/-) mice [9] and H222P knock-in mice (LmnaH222P/H222P) carrying a missense mutation identified in a family with AD-EDMD [19, 20] have been generated to elucidate the pathomechanisms of laminopathy and EDMD.