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. 2022 Apr 14;12(4):632. doi: 10.3390/jpm12040632

Figure 3.

Figure 3

Stimulation of CFTR-mediated chloride and bicarbonate transport by ELX/TEZ/IVA in epithelial monolayers (2D HIOs). (A–D) Representative Isc recordings of the effect of CFTR modulators on forskolin-induced Isc responses. (A,B) Patient 1 (E193K/F508del). (C,D) Patient 2 (R334W/F508del). Experiments were performed in medium that either contained chloride (A,C) or bicarbonate (B,D) as the single CFTR-permeating anion. Arrowheads indicate the addition of forskolin (1) and CFTRinh172 (2), respectively. (E) Aggregate data showing peak Isc responses elicited in chloride medium. (F) Aggregate data showing peak Isc responses elicited in bicarbonate medium. The horizontal, dotted line denotes the magnitude of the forskolin-induced Isc response in organoid monolayers expressing wildtype CFTR. Mean ± SD of 6 (E193K/F508del) or 3 (R334W/F508del) technical replicates are shown.