Summary
Proper development of both the intrahepatic and the extrahepatic biliary tracts is critical to the proper functions of the liver related to bile production and excretion. Many disorders that result in fibrocystic disease of the liver have, at their origin, a defect in the early development of the liver. In particular, disordered development of the ductal plate can lead to severely debilitating hepatobiliary disorders such as biliary atresia and fibrocystic disease of the liver. In this chapter, I review current knowledge of the embryological development of the extrahepatic and intrahepatic biliary trees with a focus on the genes and molecular pathways that have recently been shown to play critical roles in biliary development. Although the identification of the genetic pathways involved in biliary development is still quite incomplete, recent progress has shown that there are a number of transcription factors and signaling pathways that regulate both hepatoblast differentiation and biliary morphogenesis. Building on this exciting new knowledge will provide new avenues for both the diagnosis and the treatment of debilitating diseases that, up to this point, have no definitive treatment or cure.
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Bogue, C.W. (2010). Embryology and Development of the Ductal Plate. In: Murray, K., Larson, A. (eds) Fibrocystic Diseases of the Liver. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-524-8_1
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