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Biochim Biophys Acta Mol Basis Dis. 2018 Apr;1864(4 Pt B):1374-1379. doi: 10.1016/j.bbadis.2017.07.023. Epub 2017 Jul 25.

Pathophysiologic implications of innate immunity and autoinflammation in the biliary epithelium.

Author information

1
Digestive Disease Section, Yale University School of Medicine, New Haven, CT, USA; International Center for Digestive Health, Department of Surgery and Translational Medicine, University of Milan-Bicocca, Milan, Italy. Electronic address: mario.strazzabosco@yale.edu.
2
Digestive Disease Section, Yale University School of Medicine, New Haven, CT, USA; International Center for Digestive Health, Department of Surgery and Translational Medicine, University of Milan-Bicocca, Milan, Italy.
3
International Center for Digestive Health, Department of Surgery and Translational Medicine, University of Milan-Bicocca, Milan, Italy.
4
Department of Molecular Medicine, University of Padova School of Medicine, Padova, Italy.
5
Digestive Disease Section, Yale University School of Medicine, New Haven, CT, USA.
6
Digestive Disease Section, Yale University School of Medicine, New Haven, CT, USA; International Center for Digestive Health, Department of Surgery and Translational Medicine, University of Milan-Bicocca, Milan, Italy; Department of Molecular Medicine, University of Padova School of Medicine, Padova, Italy.

Abstract

The most studied physiological function of biliary epithelial cells (cholangiocytes) is to regulate bile flow and composition, in particular the hydration and alkalinity of the primary bile secreted by hepatocytes. After almost three decades of studies it is now become clear that cholangiocytes are also involved in epithelial innate immunity, in inflammation, and in the reparative processes in response to liver damage. An increasing number of evidence highlights the ability of cholangiocyte to undergo changes in phenotype and function in response to liver damage. By participating actively to the immune and inflammatory responses, cholangiocytes represent a first defense line against liver injury from different causes. Indeed, cholangiocytes express a number of receptors able to recognize pathogen- or damage-associated molecular patterns (PAMPs/DAMPs), such as Toll-like receptors (TLR), which modulate their pro-inflammatory behavior. Cholangiocytes can be both the targets and the initiators of the inflammatory process. Derangements of the signals controlling these mechanisms are at the basis of the pathogenesis of different cholangiopathies, both hereditary and acquired, such as cystic fibrosis-related liver disease and sclerosing cholangitis. This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen.

KEYWORDS:

Cholangiocytes; Cytokines; Inflammasome; Inflammation; Toll-like receptor

PMID:
28754453
PMCID:
PMC5785585
[Available on 2019-04-01]
DOI:
10.1016/j.bbadis.2017.07.023

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