Together, our results show that dysferlin exerts protective effects on the fukutinHp/− FCMD mouse model, and the (dysferlinsjl/sjl: fukutinHp/−) mice will be useful as a novel model for a recently proposed antisense oligonucleotide therapy for FCMD. This evidence concerns the gene DYSF and Congenital muscular dystrophy, Fukuyama type.