The following antibodies were used: mouse monoclonal anti-CFTR (ab769 and ab596, J.R. Riordan, University of North Carolina at Chapel Hill, and Cystic Fibrosis Foundation Therapeutics); mouse monoclonal anti-GAPDH (sc-32233; Santa Cruz Biotechnology, Inc.; RRID:AB_627679); horseradish peroxidase (HRP)-conjugated anti-mouse IgG (ab97023; Abcam; RRID:AB_10679675).
Vectors encoding wt-, L467F-, F508del-, and L467F-F508del-CFTR variants were purchased from VectorBuilder (vector IDs available upon request; Neu-Isenburg, Germany).
The CFTR modulators ivacaftor, tezacaftor, and lumacaftor were from TargetMol (catalog ID: T2588, T2263, and T2595, respectively; Wellesley Hills, MA, USA) while elexacaftor was purchased from MedChemExpress (catalog ID: HY-111772; Monmouth Junction, NJ, USA). The final working concentration used for the CFTR modulators were as follows: elexacaftor, 3 µM; tezacaftor, 10 µM; lumacaftor, 3 µM; ivacaftor, 1 µM (when applied acutely during short-circuit current measurements or for the YFP assay) or 5 µM (for 24 h treatment in the ETI combination).
Vectors encoding wt-, L467F-, F508del-, and L467F-F508del-CFTR variants were purchased from VectorBuilder (vector IDs available upon request; Neu-Isenburg, Germany).
The CFTR modulators ivacaftor, tezacaftor, and lumacaftor were from TargetMol (catalog ID: T2588, T2263, and T2595, respectively; Wellesley Hills, MA, USA) while elexacaftor was purchased from MedChemExpress (catalog ID: HY-111772; Monmouth Junction, NJ, USA). The final working concentration used for the CFTR modulators were as follows: elexacaftor, 3 µM; tezacaftor, 10 µM; lumacaftor, 3 µM; ivacaftor, 1 µM (when applied acutely during short-circuit current measurements or for the YFP assay) or 5 µM (for 24 h treatment in the ETI combination).