The epithelial cells lining intrahepatic bile ducts (i.e., cholangiocytes), like 
many cell types in the body, have primary cilia extending from the apical plasma 
membrane into the bile ductal lumen. Cholangiocyte cilia express proteins such 
as polycystin-1, polycystin-2, fibrocystin, TRPV4, P2Y12, AC6, that account for 
ciliary mechano-, osmo-, and chemo-sensory functions; when these processes are 
disturbed by mutations in genes encoding ciliary-associated proteins, liver 
diseases (i.e., cholangiociliopathies) result. The cholangiociliopathies include 
but are not limited to cystic and fibrotic liver diseases associated with 
mutations in genes encoding polycystin-1, polycystin-2, and fibrocystin. In this 
review, we discuss the functions of cholangiocyte primary cilia, their role in 
the cholangiociliopathies, and potential therapeutic approaches.