Development of the bile ducts: essentials for the clinical hepatologist

J Hepatol. 2012 May;56(5):1159-1170. doi: 10.1016/j.jhep.2011.09.022. Epub 2012 Jan 13.

Abstract

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Bile Duct Diseases / etiology*
  • Bile Duct Diseases / pathology
  • Bile Duct Diseases / physiopathology
  • Bile Ducts / embryology*
  • Bile Ducts / pathology
  • Bile Ducts / physiopathology
  • Epithelium / embryology
  • Epithelium / pathology
  • Epithelium / physiopathology
  • Humans
  • Liver / embryology
  • Liver / pathology
  • Liver / physiopathology
  • Liver Diseases / etiology
  • Liver Diseases / pathology
  • Liver Diseases / physiopathology
  • Morphogenesis / physiology*