ZSCAN4 promoter-based reporter gene assay detects endogenous Dux4 activity in FSHD1 myotubes.
A, luciferase activity measured in ZSCAN4 RGA–transduced healthy (Skmdc) and FSHD1 (FSHD940) myoblasts and myotubes. B, qPCR for DUX4 in Skmdc and FSHD940 myoblasts and myotubes. C, luciferase activity measured in ZSCAN4 RGA–transduced FSHD940 myotubes transfected with a nontargeting siRNA (CON) or siRNA against Dux4 (DUX4). D, qPCR for DUX4 in FSHD940 myotubes transfected with a nontargeting siRNA (CON) or siRNA against Dux4 (DUX4); **, p < 0.01. Error bars, S.E.