DUX4 and Emery-Dreifuss muscular dystrophy: In EDMD, an altered genome organization affects the gene expression [50,51,52], while in FSHD, altered methylation has been proven to be a better predictive marker than the length of repeat contraction [75], and thus the gene expression is a relevant factor on top of the altered expression of the transcription factor DUX4 [55,56].