Figure 2.

VX-809–mediated reduction in cyst formation. WT and del4 cholangiocytes were grown on Matrigel and monitored for 1 week. Cholangiocytes treated with (A, B) VX-809 or (C, D) FDL169. A smaller total cystic area was observed after treatment with either corrector. Cysts were induced with forskolin (For) (10 μM) for 24 hours on day 6. Arrows indicate WT cells. (E, F) Cholangiocytes were treated with VX-809 in the absence of forskolin. Cyst growth was monitored every other day. Cysts were treated with VX-809 (10 μM) for 10 days, with the medium was replaced every 48 hours. Number of cysts measured are represented in the figure as dots. Student’s t test. ∗P < .05; ∗∗P < .01; ∗∗∗P < .001; ∗∗∗∗P < .0001.