Experimental models of biliatresone-induced biliary atresia

Abstract

Biliary atresia (BA) is the most common cause of neonatal cholestasis and is characterized by the progressive obliteration of the extrahepatic biliary tree and by the fibro-inflammatory obstruction of the extrahepatic biliary ducts. It is the most frequent cause of liver transplantation in the pediatric population. The etiology of BA is complex with genetic and environmental factors playing an important role. A newly isolated toxin, biliatresone, has been shown to mimic the BA phenotype in mice and zebrafish. In this review, we recapitulate and compare the different experimental models where biliatresone has proved to mimic BA disease. We revisit the biliatresone action mechanism, and summarize the new discoveries on BA etiology based on biliatresone experimentation.