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. 2017 May 31;26(16):3116–3129. doi: 10.1093/hmg/ddx196

Figure 6.

Figure 6

Effects of nonsense suppression, corrector, and potentiator compounds on CFTR function restored in cells expressing CFTR G542X and W1282X alleles. (A, C) Representative tracings of Isc in FRT monolayers expressing CFTR G542X or W1282X alleles after treatment with various combinations of G418 (250 µg/ml) and VX-809 (3 µM) for 24 h and VX-770 (10 µM) for 10 min. (B, D) Summaries of Isc data following mRNA normalization. N = 12 per condition. (E, F) Summaries of ΔGt data following mRNA normalization. N = 6 per condition. See Supplementary Material, Figure S3C for representative Gt tracings. (G) Representative equivalent current (Ieq) tracings of G542X/G542X primary HBE cells treated with DMSO vehicle, G418 (100 µM) or VX-809 (3 µM), or a combination of G418 and VX-809 for 48 h. (H) Summary data expressed as AUC between activation of CFTR-dependent Ieq with forskolin (10 µM) and ivacaftor (VX-770, 1 µM) for 30 min prior to inhibition of anion transport with bumetanide (10 µM). N = 4/condition. *P < 0.05, **P < 0.01, ***P < 0.001,****P < 0.0001 versus untreated or vehicle (unless otherwise indicated).