Fig. 1.

BETi block DUX4-mediated gene expression. a The BETi (+)-JQ1 and PFI-1 block ZSCAN4 luciferase reporter activity in differentiating 54-2 FSHD1 myotubes with EC₅₀s of 50 and 210 nM, respectively. b–c Treatment with 500 nM of the BETi (+)-JQ1 prevents induction of endogenous ZSCAN4 (b) and DUX4 (c) mRNA as 54-2 FSHD1 cells differentiate from myoblasts to 6-day-old myotubes. Relative mRNA levels for each gene were normalized to that in undifferentiated myoblasts, which was set to 1. 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