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Nat Rev Gastroenterol Hepatol. 2019 May;16(5):269-281. doi: 10.1038/s41575-019-0125-y.

Cholangiocyte pathobiology.

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Department of Liver and Gastrointestinal Diseases, Biodonostia Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), CIBERehd, Ikerbasque, San Sebastian, Spain.
Division of Gastroenterology and Hepatology, Mayo School of Medicine and Science, Mayo Clinic, Rochester, MN, USA.
Institute of Clinical Medicine and Division of Surgery, Inflammatory Diseases and Transplantation, University of Oslo and Oslo University Hospital, Oslo, Norway.
Yale Liver Center, Department of Internal Medicine, Yale University, New Haven, CT, USA.
Division of Gastroenterology and Hepatology, Mayo School of Medicine and Science, Mayo Clinic, Rochester, MN, USA.


Cholangiocytes, the epithelial cells lining the intrahepatic and extrahepatic bile ducts, are highly specialized cells residing in a complex anatomic niche where they participate in bile production and homeostasis. Cholangiocytes are damaged in a variety of human diseases termed cholangiopathies, often causing advanced liver failure. The regulation of cholangiocyte transport properties is increasingly understood, as is their anatomical and functional heterogeneity along the biliary tract. Furthermore, cholangiocytes are pivotal in liver regeneration, especially when hepatocyte regeneration is compromised. The role of cholangiocytes in innate and adaptive immune responses, a critical subject relevant to immune-mediated cholangiopathies, is also emerging. Finally, reactive ductular cells are present in many cholestatic and other liver diseases. In chronic disease states, this repair response contributes to liver inflammation, fibrosis and carcinogenesis and is a subject of intense investigation. This Review highlights advances in cholangiocyte research, especially their role in development and liver regeneration, their functional and biochemical heterogeneity, their activation and involvement in inflammation and fibrosis and their engagement with the immune system. We aim to focus further attention on cholangiocyte pathobiology and the search for new disease-modifying therapies targeting the cholangiopathies.

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