A) WT and Pkhd1del4/del4 cholangiocytes were cultured over membrane inserts and labelled with a monoclonal rabbit anti E-cadherin antibody (green) and the nuclei were stained with DAPI (blue). A clear reduction and mislocalization of E-cadherin is present in Pkhd1del4/del4 cholangiocytes (magnification 400x). B) Representative Western blot and quantitative analysis illustrating a reduction of E-cadherin in Pkhd1del4/del4 compared to WT cholangiocytes. Forskolin further decreased the expression of E-cadherin in Pkhd1del4/del4 but not in WT cholangiocytes (n=4; *p<0.05 vs WT; op<0.05 vs controls). C) Gene expression of Zeb-1, a negative regulator of E-cadherin , was significantly reduced in Pkhd1del4/del4 cholangiocytes after treatment with the β-catenin inhibitor, quercetin (Q), or with the Rac-1 inhibitor, NSC 23766 (NSC). Data are normalized to untreated control (C) cells. (n=7; *p<0.05 vs controls).