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. 2019 Aug;370(2):219–230. doi: 10.1124/jpet.119.259663

Fig. 1.

Fig. 1.

p38 Inhibitor PH-797804 reduces DUX4 and DUX4 target gene expression in FSHD patient-derived proliferating myoblasts and differentiating myotubes. (A) PH-797804 concentration-response curve. Differentiating MB200 (FSHD2) cells were treated with PH-797804 in an 11-point dilution series for 40 hours. RNA levels for DUX4 target MBD3L2 and differentiation markers MYOG and MYH2 were determined in cell lysates by quantitative real-time (qRT) PCR. (B) PH-797804 in FSHD myoblasts. Proliferating cultures of 54-2 (FSHD1) and MB200 (FSHD2) myoblasts were treated with 100 nM PH-797804 for 72 hours. RNA was isolated and analyzed for DUX4 targets MBD3L2, ZSCAN4, and LEUTX. (C and D) Differentiating cultures of 54-2 (FSHD1) and MB200 (FSHD2) muscle cells were treated with varying concentrations of PH-797804, as indicated, for 40 hours and the cultures were analyzed for DUX4, DUX4 target (MBD3L2, ZSCAN4, and LEUTX), and differentiation marker (MYOG and MYH2) RNA levels by qRT-PCR. Data are expressed as relative expression (mean with S.D.) with the expression in absence of inhibitor set to 1. *P < 0.01 vs. control (unpaired, two-tailed t test). **P < 0.01 vs. control (one-way ANOVA with Dunnett’s post test).