CFTR and autosomal dominant polycystic kidney disease: A particular feature of BA that may lend itself specifically to ADPKD therapy is its expected activity limitation primarily to kidney and liver, the two organs predominantly affected in the disease, where the BA pro-drug can get converted to its active metabolite via local expression of very long-chain acyl-CoA synthetase (FATP2 or ACSVL1; Cf.