Please use this identifier to cite or link to this item: http://hdl.handle.net/20.500.12857/116501
Title: Fibroinflammatory Liver Injuries as Preneoplastic Condition in Cholangiopathies
Authors: Cannito, Stefania
Milani, Chiara 
Cappon, Andrea 
Parola, Maurizio
Strazzabosco, Mario
Cadamuro, Massimiliano 
Keywords: Caroli’s disease;cholangiocarcinoma;cholangiocytes;neoplastic transformation;primary sclerosing cholangitis
Keywords Plus: HEPATIC STELLATE CELLS;POLYCYSTIC KIDNEY-DISEASE;TISSUE GROWTH-FACTOR;PRIMARY SCLEROSING CHOLANGITIS;PRIMARY BILIARY-CIRRHOSIS;DUCTULAR REACTION;INTRAHEPATIC CHOLANGIOCARCINOMA;FACTOR-BETA;ANIMAL-MODELS;MOUSE CHOLANGIOCYTES
Mesh headings: Bile Duct Diseases;Liver;Liver Cirrhosis;Precancerous Conditions
Secondary Mesh headings: Animals;Epithelium;Humans;Mesoderm
Issue Date: 4-Dec-2018
Publisher: MDPI
Journal: International journal of molecular sciences 
Abstract: 
The cholangipathies are a class of liver diseases that specifically affects the biliary tree. These pathologies may have different etiologies (genetic, autoimmune, viral, or toxic) but all of them are characterized by a stark inflammatory infiltrate, increasing overtime, accompanied by an excess of periportal fibrosis. The cellular types that mount the regenerative/reparative hepatic response to the damage belong to different lineages, including cholagiocytes, mesenchymal and inflammatory cells, which dynamically interact with each other, exchanging different signals acting in autocrine and paracrine fashion. Those messengers may be proinflammatory cytokines and profibrotic chemokines (IL-1, and 6; CXCL1, 10 and 12, or MCP-1), morphogens (Notch, Hedgehog, and WNT/β-catenin signal pathways) and finally growth factors (VEGF, PDGF, and TGFβ, among others). In this review we will focus on the main molecular mechanisms mediating the establishment of a fibroinflammatory liver response that, if perpetuated, can lead not only to organ dysfunction but also to neoplastic transformation. Primary Sclerosing Cholangitis and Congenital Hepatic Fibrosis/Caroli's disease, two chronic cholangiopathies, known to be prodrome of cholangiocarcinoma, for which several murine models are also available, were also used to further dissect the mechanisms of fibroinflammation leading to tumor development.
URI: http://hdl.handle.net/20.500.12857/116501
ISSN: 16616596
DOI: 10.3390/ijms19123875
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