Since DUX4 expression is causal for FSHD (Lemmers et al., 2010; Snider et al., 2010), and FSHD pathology is associated with active muscle regeneration (Snider et al., 2010), we investigated whether the DUX4 regulatory elements were active during skeletal muscle regeneration. Here, DUX4 is linked to facioscapulohumeral muscular dystrophy.