Cholangiopathy: Genetics, Mechanism, and Pathology

Cholangiopathy is pathologically and pathogenetically heterogeneous and presents a broad spectrum of clinical manifestations. A majority of them are known for many years, while some are newly emerging diseases. Recent advances in biology and medicine have introduced new technologies to study the cholangiocyte biologies and physiologies and the genetics, and the pathogenesis and pathology of cholangiopathy is now being evaluated from the aspects of experimental and clinical studies. Several animal models have been developed for autoimmune and genetic cholangiopathy such as primary biliary cirrhosis and polycystic disease of the liver and biliary tract. Knowledge and understanding of these conditions have led to the development of promising therapies and novel tools to characterize these clinical conditions