Finally, given (I) the regeneration defects in FSHD [89,90,91], (II) the role of HIF1α in this process [38,40] and (III) the impact of DUX4 expression on the HIF1α pathway depending on the differentiation state, further investigations, especially in cellular actors of muscle regeneration (e.g., satellite cells) appear critical for a better understanding of FSHD-associated muscle regeneration disturbances. Here, DUX4 is linked to facioscapulohumeral muscular dystrophy.