Similarly, DUX4 derepression in muscle cells causes the human disease facioscapulohumeral muscular dystrophy (FSHD), characterized by up-regulation of DUX4 target genes, dsRNAs, TEs, and apoptosis (Dixit et al. 2007; Geng et al. 2012; Shadle et al. 2017). The gene discussed is DUX4; the disease is facioscapulohumeral muscular dystrophy.