The researchers obtained from iPSC of CFTR-F508del homozygous patients F508del cholangiocytes reproducing the polarity and the secretory function of the biliary epithelium. PKA/c-AMP mediated fluid secretion was impaired in F508del cholangiocytes and negligibly improved by VX-770 and VX-809, two small molecule drugs used to correct and potentiate ΔF508 CFTR. Moreover, ∆F508 cholangiocytes showed increased phosphorylation of SFK (Src kinase family) and TLR4 and pro-inflammatory changes. Treatment with Src inhibitor (PP2) decreased the inflammatory changes and improved cytoskeletal defects. Inhibition of SFK along with administration of VX-770 and VX-809 successfully restored fluid secretion to normal levels. The study has shown that, in regard of CFLD, targeting Src kinase and decreasing inflammation may increase the efficacy of pharmacological therapies aimed at correcting the basic ∆F508 defect. Moreover it has outlined that iPSC technology can be used to perform studies on the efficacy of new drugs for CFLD and possibly applicable to other organs (i.e. lung, pancreas, intestine).