Ectopic expression of DUX4 in human myocytes activates immune-related genes [40], and DUX4 target genes are upregulated in actively inflamed FSHD muscle biopsies, identified by magnetic resonance imaging (MRI) as short tau inversion recovery (STIR) positive [78,79]. Here, DUX4 is linked to facioscapulohumeral muscular dystrophy.