When the DUX4 is induced to be expressed at a higher level in the ACTA1-cre/FLExDUX4 mouse model, the mice develop pathological changes, including degeneration/regeneration and inflammation similar to muscle pathologies seen in the affected areas in FSHD [48,49]. The gene discussed is ACTA1; the disease is facioscapulohumeral muscular dystrophy.