Fig. 2
Effect of acute addition of correctors on calcium homeostasis of CFBE41o- F508del-CFTR cells and HAE cells. (a) Typical traces of Ca2+ response induced by the addition of 3 µM VX-809, 18 µM VX-661, or 3 µM VX-445 to the bath of CFBE41o- F508del-CFTR cells during 5 min stimulation. (b) Histogram of the normalized area under the curve (AUC) corresponding to Ca2+ mobilization in CFBE41o- F508del-CFTR cells induced by 3 µM VX-809, 18 µM VX-661, or 3 µM VX-445 (N: 2–4; n, number of cells recorded). (c) Dose-response curves for correctors on the calcium response on CFBE41o- F508del-CFTR cells. (d) Paired calcium responses of CFBE41o- F508del-CFTR cells to VX-661 (18 µM) in the presence and absence of extracellular calcium. (e) Paired calcium responses of CFBE41o- F508del-CFTR cells to VX-445 (3 µM) in the presence and absence of extracellular calcium (f) Area under the curve value with or without extracellular Ca2+ in response to correctors in CFBE41o- F508del-CFTR cells (N: 2–5; n, number of cells recorded). (g) Typical traces of Ca2+ response induced by the addition of 18 µM VX-661 in human airway epithelial (HAE; pwCF) cells during 5 min stimulation. (h) Histogram of the area under the curve (AUC) corresponding to Ca2+ mobilization induced by 18 µM VX-661 in pwCF cells (N: 4; n, number of cells recorded). (i) Typical traces of Ca2+ response induced by the addition of 18 µM VX-661 to the bath of polarized pwCF cells during 5 min stimulation. (j) Histogram of the normalized area under the curve (AUC) corresponding to Ca2+ mobilization in polarized pwCF cells induced by 18 µM VX-661 (N: 3; n, number of cells recorded). Results are presented as mean ± SEM. ****p < 0.0001