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Therapeutic Approaches for Biliary Dysgenesis of the PCK Rat, an Animal Model of Carolis Disease with Congenital Hepatic Fibrosis
Pediatrics & Therapeutics
The polycystic kidney (PCK) rat shows multiple segmental and saccular dilatations of the intrahepatic bile ducts associated with portal fibrosis, and is an orthologous rodent model of Caroli's disease with congenital hepatic fibrosis as well as autosomal recessive polycystic kidney disease. A cholangiocyte cell line that retains properties of the biliary epithelium lining the bile ducts in vivo has been developed from the PCK rat, and it has provided a novel in vitro system to study thedoi:10.4172/2161-0665.1000166 fatcat:6bvgvxxf7vhhjk5wiwhwcvcmce