Figure 7

The F508del-CFTR corrector RDR1 improves the function of the SUR1 A116P mutant. (A) Representative ⁸⁶Rb⁺ efflux profiles from COS cells transiently transfected with SUR1 A116P and wt Kir6.2 and treated with 0.1% DMSO, 300μM tolbutamide (a reversible sulfonylurea) or 10μM RDR1 as described in the METHODS. Non-transfected cells (unt) and cells transiently transfected with wt SUR1 and Kir6.2 (SURwt) served as controls. Each data point is the average of triplicates performed in the same experiment with the exception of RDR1-treated group (RDR1) in which each data point is the average of six samples. Error bars indicate S.E.M. (B) Comparison of % ⁸⁶Rb⁺ efflux at the 40-min time point in cells treated with different compounds. Only RDR1 resulted in significant improvement of channel activity. Each data point is the mean ± S.E.M. (n = 7) from 4 independent experiments; *, p < 0.05 compared to DMSO treated group by one-way ANOVA and Dunnett’s post-hoc test.