Special Issue "Molecular and Cellular Interactions in Biliary Tree Development, Diseases and Cancer"
Deadline for manuscript submissions: closed (31 August 2018).
2. Section of Digestive Diseases, Yale Liver Center (YLC), Yale University, 300 Cedar Street, New Haven, CT 06520, USA
Interests: biliary disease pathophysiology; epithelial-mesenchymal interactions; liver repair; tumor microenvironment
Primary diseases of the biliary epithelium (i.e., cholangiopathies) are a heterogeneous group of chronic liver diseases frequently affecting children or young adult individuals. Cholangiopathies are characterized by bile duct damage evolving into ductopenia, with progressive cholestasis and portal fibrosis. Although actively investigated since the early 1990s, most of them still lack effective therapies. Treatment for cholangiopathies is limited to ursodeoxycholic acid (UDCA) and obeticholic acid (OCA) in primary biliary cholangitis, and to liver transplantation in the most advanced diseases.
In cholangiopathies, the fundamental mechanism targeting the biliary epithelium is inflammation, often associated to peribiliary fibrogenesis that may progress to biliary cirrhosis and eventually malignant transformation. It is now recognized that inflammation is part of a highly-orchestrated process in which biliary epithelial cells (i.e., cholangiocytes), by reactivating a phenotype typically displayed during the embryonic development, establish with mesenchymal cells intimate contacts and mutually exchange a variety of signals. This Special Issue will highlight in both development and pathology conditions, the different settings featuring these cell interactions along with the multiple cell types involved and the paracrine signals mediating the cell communications. A range of primary cholangiopathies, from developmental (biliary atresia, congenital hepatic fibrosis, polycystic liver disease, cystic fibrosis, Alagille syndrome), to immune-mediated (primary biliary cirrhosis, primary sclerosing cholangitis) and malignant (cholangiocarcinoma) bear witness to the pathophysiological relevance of these cellular interactions.
The main goal of the studies that will be included in this Special Issue is to pinpoint the translational potential of this approach, and the identification of novel therapeutic targets to inhibit progression to cirrhosis and cancer.
Prof. Dr. Luca Fabris
Prof. Dr. Carlo Spirli
Prof. Dr. Joachim Mertens
Manuscript Submission Information
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- Endothelial cells
- NK cells
- Hepatic progenitor cells
- Ductular reaction
- Growth factors
- Primary biliary cholangitis
- Primary sclerosing cholangitis
- Biliary atresia
- Cystic Fibrosis
- Fibropolycystic liver disease