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. 2014 Sep 30;9(9):e106842. doi: 10.1371/journal.pone.0106842

Figure 5. TGF-beta downregulation of VX-809-corrected F508del CFTR in HAECs.

Figure 5

F508del CFTR homozygous HAECs were grown at air-liquid interface culture for 6 weeks and treated with TGF-beta (10 ng/ml) and/or VX-809 (3 µM) for 48 h prior to Isc measurement. (A) Stimulated Isc under control (left), VX-809 alone (middle), or VX-809 + TGF-beta (right) treatment conditions. *P<0.035, VX-809 compared with either control or VX-809 + TGF-beta. (B) Lysates of F508del HAECs from each filter were prepared and subjected to PAGE and immunoblot as described in Methods for detection of B Band and C Band under control, VX-809 alone, or VX-809 + TGF-beta treatment conditions. (C) All tested doses of TGF-beta downregulated VX-809-corrected F508del CFTR. *P<0.00017, VX-809 compared with untreated control; **P<0.0001, 0.1 ng TGF-beta + VX-809 compared with VX-809 alone. ***P<0.0001, 1 ng TGF-beta + VX-809 compared with VX-809 alone. ****P<0.0001, 10 ng TGF-beta + VX-809 compared with VX-809 alone. (D) Summary densitometry data for detection of F508del CFTR B Band (left) and C Band (right) with and without TGF-beta (10 ng/ml, similar to 5B). *P≤0.047, B Band VX-809 compared with either control or VX-809 + TGF-beta. *P≤0.05, C Band VX-809 compared with either control or VX-809 + TGF-beta.