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. 2013 Sep;49(3):403–409. doi: 10.1165/rcmb.2013-0100OC

Figure 5.

Figure 5.

Effects of ataluren readthrough on the proliferation of PAECs. Live cell counts show that ataluren treatment leads to growth suppression of R321X (A) and restores BMP-mediated growth repression in response to BMP9 (B) (post hoc P values versus control at 72-h: R321X + ataluren, P < 0.001; R294X + ataluren + BMP9 versus control + BMP9, P = 0.42). In cell proliferation assays using XTT: (C) untreated PAECs from heritable PAH (HPAH) proliferate significantly faster than controls (*post hoc pairwise P values at 72 h; all P < 0.001); (D) BMP9 treatment slows proliferation in all cells, but HPAH cases still proliferate faster than untreated control cells (*P < 0.001); (E) Ataluren treatment significantly represses the growth of R321X and R294X cells, but has no effect on Δex1–8 cells (*P < 0.001); (F) BMP9 induction after ataluren treatment shows restoration of BMP growth repression in R321X and R294X, but not Δex1–8 cells. Means (±SD) of triplicate wells are shown.