Newer variants of progressive familial intrahepatic cholestasis

Figure 2 Diagrammatic representation of interaction between various tight junction proteins in hepatocytes. Claudin, tight junction proteins (TJP2), and actin form intercellular cytoskeletal support. Tight junctions prevent mixing of bile and blood. Absence of TJP2 causes a failure of claudin-1 localization at the canalicular membrane, leading to loss of compactness of the tight junctions and leakage of the bile through the paracellular space. TJP2: Tight junction proteins 2.