Tissue Culture Correlational Study of Genetic Cholangiopathy of Autosomal Recessive Polycystic Kidney Disease

摘要: Cholangiocytes are epithelial cells that line the biliary tract and also known as (BECs). In vitro culture studies of BECs in correlation with tissue section examination may give us a comprehensive analysis diseases. Herein, we discuss genetic cholangiopathy autosomal recessive polycystic kidney disease (ARPKD), mainly using (PCK) rat, an animal model ARPKD. The hepatobiliary lesions ARPKD patients (Caroli's congenital hepatic fibrosis) PCK rats speculated to be related mutations 1 (PKHD1) which have been recently demonstrated, though exact causal relation between these pathology remain clarified. Recently clarified rat showed increased cell proliferation followed by irregular dilatation intrahepatic bile ducts. We identified essential involvement MEK5-ERK5 pathway abnormal rat. degradation laminin type IV collagen (basal membrane components ducts) was closely dysgenesis cystogenesis rats. mesenchymal phenotype progressive portal fibrosis, indicating TGF-β1 involved this acquisition phenotype. Detailed mandatory evaluate pathogenesis cholangiopathy.