Journal
MOLECULAR ASPECTS OF MEDICINE
Volume 29, Issue 1-2, Pages 50-57Publisher
ELSEVIER
DOI: 10.1016/j.mam.2007.09.007
Keywords
PBC; cholangiocarcinoma; cholangiopathies; ADPKD; cholangiocytes; estrogens; IGF1
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Cholangiopathies are diseases of high social impact representing the main indication for liver transplantation in the infanthood and the third in adulthood. Despite the heterogeneous etiology and pathogenesis, cholangiopathies share many different common morphological features and, chronically progress toward a ductupenic condition clinically evidenced by the classical features of a cholestatic syndrome. The primary target of damage in the course of cholangiopathies are cholangiocytes, the epithelia cells lining the biliary tree. A bulk of researches performed in the last decade, highlighted the extraordinary biological properties of cholangiocytes involved in a number of important processes such as bile formation, proliferation, injury repair, fibrosis, angiogenesis and regulation of blood flow. Recent advances on the molecular and cell biology of human cholangiopathies are opening new potential therapeutic perspectives for these diseases. (C) 2007 Elsevier Ltd. All rights reserved.
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