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Special Issue "Molecular and Cellular Interactions in Biliary Tree Development, Diseases and Cancer"
Deadline for manuscript submissions: closed (31 August 2018).
Prof. Dr. Luca Fabris E-Mail
1. Department of Molecular Medicine (DMM), University of Padua School of Medicine, Viale G. Colombo, 3, 35131 Padua, Italy
2. Section of Digestive Diseases, Yale Liver Center (YLC), Yale University, 300 Cedar Street, New Haven, CT 06520, USA
Prof. Dr. Carlo Spirli E-Mail
Section of Digestive Diseases, Yale Liver Center (YLC), Yale University, 300 Cedar Street, New Haven, CT 06520, USA
Prof. Dr. Joachim Mertens E-Mail
Clinic of Gastroenterology and Hepatology, University-Hospital of Zurich, Rämistrasse 100, 8091 Zürich, Switzerland
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
Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All papers will be peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.
Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.
Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.
- Endothelial cells
- NK cells
- Hepatic progenitor cells
- Ductular reaction
- Growth factors
- Primary biliary cholangitis
- Primary sclerosing cholangitis
- Biliary atresia
- Cystic Fibrosis
- Fibropolycystic liver disease