Fig. 4

Beta-2 agonists inhibit DUX4-mediated gene expression. a–g Expression of DUX4, DUX4 target genes, and the myogenic markers MYOG or CKM in MB073 FSHD1 (a, d, f) or MB200 FSHD2 (b, c, e, g) myoblasts (a–b) or myotubes (c–g). Myoblasts were treated with 1 nM formoterol or DMSO vehicle control for 17 h. Myotubes were differentiated for 48 h with 1 nM formoterol or DMSO added during the final 24 h (c); the final 8, 4, 2, or 1 h (d–e); or the final 8 h after which compound was washed out and cells harvested 16 h later (f–g). Error bars indicate the standard deviation from the mean of three biological replicates. p values were calculated using a two-tailed, two-sample Student’s t test assuming unequal variance. *p < 0.05