Pathophysiologic implications of innate immunity and autoinflammation in the biliary epithelium |
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Authors: | Mario Strazzabosco Romina Fiorotto Massimiliano Cadamuro Carlo Spirli Valeria Mariotti Eleanna Kaffe Roberto Scirpo Luca Fabris |
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Institution: | 1. Digestive Disease Section, Yale University School of Medicine, New Haven, CT, USA;2. International Center for Digestive Health, Department of Surgery and Translational Medicine, University of Milan-Bicocca, Milan, Italy;3. Department of Molecular Medicine, University of Padova School of Medicine, Padova, Italy |
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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. |
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Keywords: | RDC reactive ductular cell DAMPs danger associated molecular patterns PAMPs pathogen associated molecular patterns cAMP cyclic adenosine monophosphate NOS nitric oxide synthase RNOS reactive nitrogen oxide species CFTR cystic fibrosis transmembrane conductance regulator AE2 anion exchange protein 2 LPS lipopolysaccharide PSC primary sclerosing cholangitis TLR Toll-like receptor hBD2 human beta defensing 2 NR nuclear receptor MyD88 myeloid differentiation primary response gene 88 Mal MyD88 adapter like TRIF TIR-domain-containing adapter-inducing interferon-β TRAM TRIF-related adaptor molecule G-CSF granulocyte-colony stimulating factor LIX lipopolysaccharide-induced CXC chemokine HMGB-1 high mobility group box 1 protein ATP adenosine triphosphate HSP heat shock protein PPAR-γ peroxisome proliferator-activated receptor gamma VDR vitamin D receptor FXR farnesoid X receptor GR glucocorticoid receptor CHF congenital hepatic fibrosis PKHD1 polycystic kidney and hepatic disease 1 FPC fibrocystin CXCL1 chemokine ligand 1 CXCL10 C-X-C motif chemokine 10 CXCL12 C-X-C motif chemokine 12 IBD inflammatory bowel disease Nlrp3 NLR family pyrin domain containing 3 iPSC induced pluripotent stem cells Cholangiocytes Inflammation Cytokines Toll-like receptor Inflammasome |
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