Peter van Mourik

34 Chapter 2 Supplementary table 3. Overview of correlation analyses performed in this study. Variable 1 Variable 2 Pearson’s r p-value R117H-CFTR mRNA expression CFTR protein expression 0.92 <0.0001 R117H-CFTR mRNA expression in compound heterozygous R117H cultures CFTR protein expression in compound heterozygous R117H cultures 0.88 <0.0001 R117H-CFTR mRNA expression 0.128 μm forskolin-induced swelling 0.75 <0.01 R117H-CFTR mRNA expression 0.05 μm forskolin-induced swelling 0.57 0.04 R117H-CFTR mRNA expression 0.128 μm forskolin-induced swelling + 3 μm VX-770 0.57 0.03 R117H-CFTR mRNA expression 0.05 μm forskolin-induced swelling + 3 μm VX-770 0.72 <0.01 R117H-CFTR mRNA expression 0.128 μm forskolin-induced swelling + 3 μm VX-770 corrected for vehicle-treated organoid swelling -0.07 NS R117H-CFTR mRNA expression 0.05 μm forskolin-induced swelling + 3 μm VX-770 corrected for vehicle-treated organoid swelling 0.69 <0.01 CFTR protein expression 0.128 μm forskolin-induced swelling 0.74 <0.01 CFTR protein expression 0.05 μm forskolin-induced swelling 0.56 0.04 CFTR protein expression 0.128 μm forskolin-induced swelling + 3 μm VX-770 0.6 0.02 CFTR protein expression 0.05 μm forskolin-induced swelling + 3 μm VX-770 0.72 <0.01 CFTR protein expression 0.128 μm forskolin-induced swelling + 3 μm VX-770 corrected for vehicle-treated organoid swelling -0.05 NS CFTR protein expression 0.05 μm forskolin-induced swelling + 3 μm VX-770 corrected for vehicle-treated organoid swelling 0.69 <0.01