The role of CFTR in the biliary tract

Cystic fibrosis (CF) is a genetic disease that leads to abnormal function of an anion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR). In the past, CF was a pediatric disease; the development of therapies has allowed people with CF to live longer. CFTR is expressed in many organs. Tissues that highly express CFTR are the bile ducts in the liver and the gallbladder; together, these tissues are referred to as the biliary tract. Disease in the liver and gallbladder is the third leading cause of death for people with CF. In this dissertation, we sought to better understand CF disease in the biliary tract through two methods: 1) using a pig model of CF that closely resemble human disease; 2) using organoids, a cell culture technique that creates a representation of an organ in a dish. Using these techniques, we found that CFTR may be important for biliary tract fluid secretion and clearance.