Disruption of planar cell polarity activity leads to developmental biliary defects

► Inhibition of planar cell polarity (PCP) genes leads to developmental biliary defects. ► Inhibition of prickle1a also leads to abnormal positioning of digestive organs. ► Biliary defects caused by prickle1a inhibition are upstream of vhnf1. ► Syndromic biliary defects may be linked to inhibition of PCP.