Regardless of whether the levels of FRG1 are increased or not in FSHD patients, the H-FRG1TG mouse is a valuable tool for studying the mechanics of muscular dystrophy, though it is important to note that FRG1 expression in these mice is many fold higher than observed in any human patients. This evidence concerns the gene FRG1 and facioscapulohumeral muscular dystrophy.