Fractalkine and other chemokines in primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is characterized by the autoimmune injury of small intrahepatic bile duct. On this basis, it has been suggested that the targeted biliary epithelial cells (BEC) play an active role in the perpetuation of autoimmunity by attracting immune cells via chemokine secretion. To address this issue, we challenged BEC using multiple toll-like receptor (TLR) ligands as well as autologous liver infiltrating mononuclear cells (LMNC) with subsequent measurement of BEC phenotype and chemokine production and LMNC chemotaxis by quantifying specific chemokines, specially CX3CL1 (fractalkine). We submit the hypothesis that BEC are in fact the innocent victims of the autoimmune injury and that the adaptive immune response is critical in PBC.