Additional file 4: Figure S2. of BET bromodomain inhibitors and agonists of the beta-2 adrenergic receptor identified in screens for compounds that inhibit DUX4 expression in FSHD muscle cells

The DUX4-responsive ZSCAN4 reporter system used for screening epigenetic modifier compounds. (A) Schematic depicting the ZSCAN4 promoter luciferase reporter vector (top) that includes four tandem DUX4 binding sites (D4BS), and a control reporter in which three of the four D4BS have been mutated (bottom). (B) Activity of the ZSCAN4 reporter vectors in 54-2 FSHD1 and 54-6 control (non-FSHD) myoblasts and 6Â day differentiated myotubes. Error bars indicate the standard deviation from the mean of three biological replicates. (PDF 66Â kb)