Immunohistochemical profile of ezrin and radixin in human liver epithelia during fetal development and pediatric cholestatic diseases.

Summary Aim Ezrin and radixin are actin-binding proteins that contribute to the integrity of epithelia. Abnormalities of bile secretion occur primarily in cholestatic liver diseases and are associated with changes in cell cytoskeleton. Expression of these proteins during liver development and in cholestatic liver diseases remains poorly investigated. Methods Ezrin and radixin expression was analyzed in fetal, adult and pediatric cholestatic human liver (i.e. biliary atresia, sclerosing cholangitis) by immunohistochemistry. Results In adult and fetal livers, ezrin was expressed exclusively in the cells of the biliary lineage (i.e. biliary epithelial cells and ductal cells) whereas radixin was located not only in hepatocytes but also in cells of the biliary lineage. In the lobule of mature livers, radixin displayed a zonal distribution with predominant expression in the periportal region. In cholestatic diseases, both proteins were expressed in cells of the ductular reaction. An aberrant expression of ezrin was detected in hepatocytes of cirrhotic nodules with a CK7-positive pattern and in malignant hepatocytes in a course of cholestatic disease toward cancer. Conclusions Among the components of the liver epithelial cells, ezrin was exclusively expressed in biliary phenotype cells, while radixin was found in biliary and hepatocytic lineages, with a periportal zonal expression. In cholestatic diseases, ezrin was expressed in hepatocytes supporting the appearance of a biliary phenotype.