It has also been shown that FSHD patient-derived cells express high levels of DUX4 during a muscle progenitor phase characterised by high levels of PAX3 and PAX7 during in vitro differentiation of embryonic stem (ES) cells, providing another time point in FSHD myogenic differentiation where competitive inhibition may occur25. Here, PAX3 is linked to facioscapulohumeral muscular dystrophy.