Preclinical insights into cholangiopathies: disease modeling and emerging therapeutic targets.

Authors:
Keisaku Sato
Keisaku Sato
Massey University
Shannon Glaser
Shannon Glaser
College of Medicine
United States
Lindsey Kennedy
Lindsey Kennedy
College of Medicine
United States
Suthat Liangpunsakul
Suthat Liangpunsakul
Indiana University School of Medicine
India
Fanyin Meng
Fanyin Meng
College of Medicine
United States
Heather Francis
Heather Francis
College of Medicine
United States
Gianfranco Alpini
Gianfranco Alpini
College of Medicine
United States

Expert Opin Ther Targets 2019 Jun 22;23(6):461-472. Epub 2019 Apr 22.

a Indiana Center for Liver Research, Division of Gastroenterology & Hepatology, Department of Medicine , Indiana University School of Medicine , Indianapolis , IN , USA.

Introduction: The common predominant clinical features of cholangiopathies such as primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), and biliary atresia (BA) are biliary damage/senescence and liver fibrosis. Curative therapies are lacking, and liver transplantation is the only option. An understanding of the mechanisms and pathogenesis is needed to develop novel therapies. Previous studies have developed various disease-based research models and have identified candidate therapeutic targets. Areas covered: This review summarizes recent studies performed in preclinical models of cholangiopathies and the current understanding of the pathophysiology representing potential targets for novel therapies. A literature search was conducted in PubMed using the combination of the searched term 'cholangiopathies' with one or two keywords including 'model', 'cholangiocyte', 'animal', or 'fibrosis'. Papers published within five years were obtained. Expert opinion: Access to appropriate research models is a key challenge in cholangiopathy research; establishing more appropriate models for PBC is an important goal. Several preclinical studies have demonstrated promising results and have led to novel therapeutic approaches, especially for PSC. Further studies on the pathophysiology of PBC and BA are necessary to identify candidate targets. Innovative therapeutic approaches such as stem cell transplantation have been introduced, and those therapies could be applied to PSC, PBC, and BA.

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http://dx.doi.org/10.1080/14728222.2019.1608950DOI Listing
June 2019
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