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. 2022 Aug 3;5(12):e202101320. doi: 10.26508/lsa.202101320

Figure S6. Comparison HC versus cystic fibrosis (CF) nasal-airway organoids (NAOs) cultured with NR/IL-1β and validation of VX-809/VX-770 responses.

Figure S6.

(A) Brightfield images (top) and images of calcein green AM–stained (bottom) NAOs cultured with NR/IL-1β, showing forskolin-induced swelling (FIS) in HC and CF F508del/F508del cultures. FIS of CF NAOs was determined in combination with vehicle or VX-809/VX-770. Images were taken at t = 0 and 120 min. (B) VX-809/VX-770 modulator response measurement in NAOs of individual donor (n = 7) with a CF F508del/F508del genotype. (C) Repeated measurements of VX-809/VX-770 modulator responses in CF F508del/F508del NAOs (n = 1 independent donor) derived from air–liquid interface cultures from serial passaged human nasal epithelial cell at P = 4, P = 5, and P = 6, using cryopreserved cells from two different vials. (D) CF F508del/F508del NAOs (n = 1 independent donor) were pre-treated with VX-809 or the CFTR correctors C1-18 for 48 h. Afterward, FIS was measured after stimulation with forskolin (Fsk) and VX-770. Vehicle was used as the control. FIS assay results are depicted as area-under-the-curve (AUC) plots (t = 120 min), means ± SD. Individual datapoints represent technical replicates.