Please use this identifier to cite or link to this item: http://hdl.handle.net/20.500.12857/116495
Title: Animal models of cholestasis: An update on inflammatory cholangiopathies
Authors: Mariotti, Valeria 
Cadamuro, Massimiliano 
Spirli, Carlo
Fiorotto, Romina
Strazzabosco, Mario
Fabris, Luca
Keywords: Biliary fibrosis;Cholangiocyte;Ductular reaction;Primary biliary cholangitis;Primary sclerosing cholangitis
Keywords Plus: PRIMARY SCLEROSING CHOLANGITIS;PRIMARY BILIARY-CIRRHOSIS;MDR2 P-GLYCOPROTEIN;AUTOIMMUNE CHOLANGITIS;MOUSE MODEL;LYMPHOPROLIFERATIVE DISORDER;MOLECULAR-MECHANISMS;ADENYLYL-CYCLASE;HCO3-UMBRELLA;RAT MODEL
Mesh headings: Cholangitis;Cholestasis;Disease Models, Animal
Secondary Mesh headings: Animals;Bile Ducts;Humans
Issue Date: 2019
Publisher: ELSEVIER SCIENCE BV
Journal: Biochimica et biophysica acta. Molecular basis of disease 
Abstract: 
Cholestasis is a frequent clinical condition initiating or complicating chronic liver diseases, particularly cholangiopathies, where the biliary epithelium is the primary target of the pathogenetic sequence. Until a few decades ago, understanding of cholestasis relied mostly on the experimental model of bile duct ligation in rodents. However, a simple model of biliary obstruction cannot reproduce the complex mechanisms and networks leading to cholestasis in cholangiopathies. These networks are underpinned by an intricate dysregulation of pro-inflammatory and pro-fibrotic signals involving besides cholangiocytes, multiple cell elements of both innate and adaptive immunity. Therefore, in the last years, a wide range of animal models of biliary injury have been developed, mostly in mice, following three main approaches, chemical induction, immunization and genetic manipulation. In this review, we will give an update of the animal models of the two main cholangiopathies, primary sclerosing cholangitis and primary biliary cholangitis, which have provided us with the most relevant insights into the pathogenesis of these still controversial diseases.
URI: http://hdl.handle.net/20.500.12857/116495
ISSN: 09254439
DOI: 10.1016/j.bbadis.2018.07.025
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