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肝内外胆管发育:先天性疾病理解的框架。

Development of the Intrahepatic and Extrahepatic Biliary Tract: A Framework for Understanding Congenital Diseases.

机构信息

de Duve Institute, Université Catholique de Louvain, 1200 Brussels, Belgium; email:

出版信息

Annu Rev Pathol. 2020 Jan 24;15:1-22. doi: 10.1146/annurev-pathmechdis-012418-013013. Epub 2019 Jul 12.

Abstract

The involvement of the biliary tract in the pathophysiology of liver diseases and the increased attention paid to bile ducts in the bioconstruction of liver tissue for regenerative therapy have fueled intense research into the fundamental mechanisms of biliary development. Here, I review the molecular, cellular and tissular mechanisms driving differentiation and morphogenesis of the intrahepatic and extrahepatic bile ducts. This review focuses on the dynamics of the transcriptional and signaling modules that promote biliary development in human and mouse liver and discusses studies in which the use of zebrafish uncovered unexplored processes in mammalian biliary development. The review concludes by providing a framework for interpreting the mechanisms that may help us understand the origin of congenital biliary diseases.

摘要

胆道在肝脏疾病的病理生理学中的作用,以及人们对胆管在肝脏组织生物构建中的再生治疗中的重视,推动了对胆管发育基本机制的深入研究。在这里,我回顾了驱动肝内和肝外胆管分化和形态发生的分子、细胞和组织机制。本综述重点讨论了促进人类和小鼠肝脏胆管发育的转录和信号模块的动力学,并讨论了利用斑马鱼揭示哺乳动物胆管发育中未被探索过程的研究。本综述最后提出了一个解释机制的框架,这可能有助于我们理解先天性胆道疾病的起源。

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