Pathogenesis of biliary atresia-Interaction between biliary innate immunity and acquired immunity-

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Biliary innate immunity for double-stranded RNA virus was examined in the histopathogenesis of biliary atresia (BA). Epithelial-mesenchymal transition (EMT) of biliary epithelial cells induced by the stimulation with double-stranded RNA and EMT-like phenotypic changes of extrahepatic bile ducts in BA are closely associated with the histogenesis of sclerosing obliterative cholangitis. Moreover, the increased number of an unique subset of NK cells, CD56^-CD16^+NK cells was found in portal tracts of BA, suggesting that NK dysfunction against virus infection might be a host factor associated with pathogenesis of BA.