FIGURE 3

Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation rescue induced by modulators. Immunoblot analysis of expression levels and molecular mass of CFTR protein, in the different control or drug-treated cystic fibrosis (CF)-“culture reprogramming condition” (CRC)-derived air–liquid interface (ALI)-differentiated samples, are shown. Nucleolin is shown for equal loading. a) F508del/F508del homozygous genotype; b), c) and d) F508del/ins (ins: one of the rare alleles with the insertion of variable length) compound heterozygotes, with genotypes according to table 1. For each panel, the densitometric quantification (mean±sd) is shown below the immunoblot. The treatments by the drugs are indicated at the bottom. LUM: lumacaftor; TEZ: tezacaftor; ELE: elexacaftor; band C: mature CFTR; band B: immature CFTR. Mean±sd of three independent experiments is shown. *: p<0.05, **: p<0.01.