Peter van Mourik

23 R117H function and VX-770 response in organoids the contribution of the R117H-homozygous organoid measurements to the observed correlation the analysis was repeated without including this data and still revealed a strong correlation (r=0.88, p<0.0001, Supplementary figure 1A). Figure 1. Forskolin-induced swelling (FIS) of R117H-CFTR organoids. (A) Representative confocal images of calcein green-labelled and forskolin-induced swelling of organoids harbouring the R117H-CFTR muta- tion demonstrating differences in FIS and treatment responses. Organoids of culture 1 have a clear lumen at t = 0 min, while organoids of culture 6 do not have a visible lumen at t = 0 min. Upon VX-770 treatment FIS occurs in both cultures. (B) AUC quantification of 0.05 µM and 0.128 µM forskolin-induced organoid swelling after 60 min. (C) AUC quantification of 0.05 µM and 0.128 µM forskolin-induced organoid swelling after 60 min of organoids treated with 3 µM VX-770. 2