Activation of the unfolded protein response (UPR) and fibrosis is associated withcholangiocellular injury in an experimental model of fibropolycystic liver disease

Background&Aim Fibropolycistic liver disease is characterized by hyperproliferation of the biliary epithelium and the formation of multiple dilated cysts, a process recently associated with unfolded protein response (UPR). In the present study, we aimed to understand the mechanisms of cyst formation and UPR activation of hepatocyte-specific c-Jun N-terminal kinase 1/2 (Jnk1/2) knockout mice.