Figure 3.

Pharmacological correction of CFTR function in W57G/A234D rectal OGs measured by the FIS assay

(A) Representative bright-field images of W57G/A234D organoids stimulated with 0.128 μM of FSK with or without CFTR modulator treatment, as indicated.

(B) Quantification of rectal OGs swelling preincubated for 24 h with the vehicle (DMSO 0.1% v/v) and the CFTR correctors (VX809-661-445 individually or in combination as indicated). At the time of the assay, rectal OGs were stimulated with various concentrations of forskolin (data selected from 60 min at 0.128 μM forskolin +VX770 3 μM). Black bars represent the value of steady-state total organoid area (SOA) before the addition of forskolin. White bar is the total organoid area after addition of forskolin (FIS assay). Ordinary one-way ANOVA: ∗< 0.05; ∗∗p < 0.002; ∗∗∗p < 0.0002; ∗∗∗∗p < 00001.