title: Molecular mechanisms of bile duct development.
abstract: The mammalian biliary system, consisting of the intrahepatic and extrahepatic bile ducts, is responsible for transporting bile from the liver to the intestine. Bile duct dysfunction, as is seen in some congenital biliary diseases such as Alagille syndrome and biliary atresia, can lead to the accumulation of bile in the liver, preventing the excretion of detoxification products and ultimately leading to liver damage. Bile duct formation requires coordinated cell-cell interactions, resulting in the regulation of cell differentiation and morphogenesis. Multiple signaling molecules and transcription factors have been identified as important regulators of bile duct development. This review summarizes recent progress in the field. Insights gained from studies of the molecular mechanisms of bile duct development have the potential to reveal novel mechanisms of differentiation and morphogenesis in addition to potential targets for therapy of bile duct disorders.
authors.affiliation: Department of Medicine, Abramson Family Cancer Research Institute, University of Pennsylvania, School of Medicine, Philadelphia, PA, USA.
authors.forename: Yiwei
authors.initials: Y
authors.lastname: Zong
authors.forename: Ben Z
authors.initials: BZ
authors.lastname: Stanger
mesh_terms: D000818:Animals; D017734:Bile Ducts, Extrahepatic / Q000196:embryology* / Q000254:growth & development*; D001653:Bile Ducts, Intrahepatic / Q000196:embryology* / Q000254:growth & development*; D001660:Biliary Tract Diseases / Q000378:metabolism / Q000473:pathology; D002454:Cell Differentiation; D018507:Gene Expression Regulation, Developmental; D022781:Hepatocytes / Q000166:cytology; D006801:Humans; D035683:MicroRNAs / Q000502:physiology; D009024:Morphogenesis; D051880:Receptors, Notch / Q000502:physiology; D015398:Signal Transduction; D016212:Transforming Growth Factor beta / Q000502:physiology; D051153:Wnt Proteins / Q000502:physiology; D051176:beta Catenin / Q000502:physiology
chemical_list: D035683:MicroRNAs; D051880:Receptors, Notch; D016212:Transforming Growth Factor beta; D051153:Wnt Proteins; D051176:beta Catenin