BILIARY ATRESIA CANDIDATE GENE Pkd1l1 IS ESSENTIAL FOR MAINTENANCE OF BILIARY EPITHELIAL CELL INTERACTION AND INFLAMMATORY RESPONSE
<div><p><b>Background:<span> </b>Biliary atresia (BA) is the most common reason for liver transplantation in infants, but its etiopathology is still not completely understood. A nationwide genomic study identified mutations in <i>PKD1L1</i> as a BA candidate gene. Liver-specific deletion of <i>Pkd1l1 </i>in<i> </i>mice results in liver pathology analogous to changes seen in BA, but how <i>Pkd1l1</i>-deficient cholangiocytes differ from wild type cells is unknown.