Development of humanized-liver mice using hepatocyte derived from pediatric liver/biliary tract diseases and investigation of pathological mechanism of liver/biliary tract diseases.

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In this study, we tried for development of humanized-liver mice in which the liver is reconstitute with human hepatocyte derived from some liver/biliary tract diseases. We demonstrated that we could produce some humanized-liver mice which have disease derived hepatocyte. Especially, we tried for producing humanized-liver using hepatocyte derived from biliary atresia. We showed that hepatocyte derived from biliary atresia produced human albumin and expressed drug metabolism enzymes in mouse liver. These results indicated that hepatocytes have normal hepatic functions even in biliary atresia. The results of this study indicated the possibility for development of humanized-disease liver mice and these mice may be useful for investigating pathological mechanism of liver/biliary tract diseases.