Department of Medicine, Abramson Family Cancer Research Institute, University of Pennsylvania, School of Medicine, Philadelphia, PA, USA.
Int J Biochem Cell Biol. 2011 Feb;43(2):257-64. doi: 10.1016/j.biocel.2010.06.020. Epub 2010 Jul 1.
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.
哺乳动物的胆道系统包括肝内和肝外胆管，负责将胆汁从肝脏输送到肠道。一些先天性胆道疾病，如 Alagille 综合征和胆道闭锁，会导致胆管功能障碍，使胆汁在肝脏中积聚，阻止解毒产物的排泄，最终导致肝脏损伤。胆管形成需要细胞间的协调相互作用，从而调节细胞分化和形态发生。已经确定了多种信号分子和转录因子作为胆管发育的重要调节剂。本综述总结了该领域的最新进展。对胆管发育分子机制的研究获得的见解除了为胆管疾病的治疗提供潜在靶点外，还有可能揭示分化和形态发生的新机制。
