Mutations in genes encoding cholangiocyte ciliary-associated proteins, such as polycystin-1 and -2 and fibrocystin, cause biliary dysgenesis, hepatic fibrosis and cystogenesis in liver diseases such as Autosomal Dominant Polycystic Kidney Disease (ADPKD) and Autosomal Recessive PKD (ARPKD). Here, PKHD1 is linked to Hepatic fibrosis.