Acting under the assumption that overexpression of FRG1, FRG2 or ANT1 plays a causative role in the development of FSHD, transgenic mice were generated expressing each of these individual genes under the human skeletal actin promoter, specific for expression in muscle, which resulted in the identification of a potential mouse model for FSHD. This evidence concerns the gene FRG2 and facioscapulohumeral muscular dystrophy.