Figure 5.
Functional characterization of W57G/A234D CFTR by short-circuit current experiments
(A) Rectal OGs growing as 2D monolayer. The data are expressed as Delta Isc values (based on maximal fsk response) of different treatments (p was calculated with the Kruskal-Wallis test).
(B) Delta Isc values expressed as μA/cm2 of monolayers of CRC-differentiated nasal cells. Cells were treated with or without VX809 (3 μM), VX661 (3 μM), VX445 (2 μM), and combinations as indicated for 24 h with DMSO as the mock control.
(C) Spearman correlation of the Delta Isc values measured in the same samples present in panels A and B showing an excellent correlation among measure values in the two models. Dashed pink line represents best-fit values. The two black dotted lines represent 95% confidence interval of the best-fit line.
(D) Representative tracings of monolayers derived from rectal OGs and CRC-differentiated nasal cells (A: Amiloride 100 μM F1: FSK 1 μM, I: VX770 1μM, F10: FSK 10 μM, In: PPQ-102 20 μM (rectal OGs) or Inh-172 (nasal cells) both at 10 μM). Isc values from WT and corrected-F508del and not corrected-F508del were used here as reference.