Development of the bile ducts: Essentials for the clinical hepatologist

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• 作者：Mario Strazzabosco¹ ; ² ; ^{mario.strazzabosco@yale.edu} ; ^{mario.strazzabosco@unimib.it} ; Luca Fabris² ; ³
• 关键词：GW ; week of gestation ; Sox9 ; SRY-related HMG box transcription factor 9 ; K ; cytokeratin ; HNF ; hepatocyte nuclear factor ; TGF ; transforming growth factor ; T尾RII ; transforming growth factor receptor type II ; PCP ; planar cell polarity ; IL ; interleukin ; Hh
• 刊名：Journal of Hepatology
• 出版年：2012
• 期刊代码：162_01688278
• 类别：med
• 出版时间：May, 2012
• 卷：56
• 期：5
• 页码：1159-1170
• 文件大小：2041 K

摘要

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Summary

Several cholangiopathies result from a perturbation of developmental processes. Most of these cholangiopathies are characterised by the persistence of biliary structures with foetal configuration. Developmental processes are also relevant in acquired liver diseases, as liver repair mechanisms exploit a range of autocrine and paracrine signals transiently expressed in embryonic life. We briefly review the ontogenesis of the intra- and extrahepatic biliary tree, highlighting the morphogens, growth factors, and transcription factors that regulate biliary development, and the relationships between developing bile ducts and other branching biliary structures. Then, we discuss the ontogenetic mechanisms involved in liver repair, and how these mechanisms are recapitulated in ductular reaction, a common reparative response to many forms of biliary and hepatocellular damage. Finally, we discuss the pathogenic aspects of the most important primary cholangiopathies related to altered biliary development, i.e. polycystic and fibropolycystic liver diseases, Alagille syndrome.