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. 2019 Sep 17;9:13460. doi: 10.1038/s41598-019-49921-4

Figure 10.

Figure 10

Efficacy of potentiation by 1 µM VX-770 of WT-, F508del-, P67L-, and G551D-CFTR expressed in FRT cells by 1 µM VX-770 is inversely proportional to the phosphorylation level. (A) Summary data showing a dose-response curve for forskolin (FSK) in FRT cells expressing WT-CFTR. n = 4. (B,C) Representative current traces of WT-CFTR activated by high and low FSK. Activation of FRT cells expressing WT-CFTR using 10 µM FSK led to near maximal currents; subsequent treatment with VX-770 led to a potentiation ratio of 0.5 ± 0.03 (Fractional increase = Ipost/Ipre − 1; Ipost, with VX-770; Ipre, without VX-770). Channels activated with 10 nM FSK were potentiated by 4.6 ± 0.15 fold. Representative current traces of G551D- (D) and P67L-CFTR (E,F) are shown. INH172, 10 µM CFTRinh172. (G) VX-770 potentiated WT-, as well as three CFTR variants in a manner variably dependent upon FSK concentration. WT-CFTR: P < 0.05, comparing 10 µM FSK vs 10 nM FSK; P < 0.05, 10 µM vs 100 nM. G551D-CFTR: P < 0.05, 10 µM vs 10 nM. F508del-CFTR: P < 0.05, 10 µM vs 10 nM. P67LCFTR: P < 0.05, 10 µM vs 10 nM. n = 5 for WT-CFTR. n = 3 each for F508del-, G551D-, and P67L-CFTR.