The molecular mechanisms underlying (hereditary) fibropolycystic diseases of the liver are still incompletely understood, although germline mutations have been identified which are associated with specific forms such as mutations of the genes Pkd1 and Pkd2 in autosomal dominant polycystic kidney disease (ADPKD) (30, 32, 33). This evidence concerns the gene PKD2 and autosomal dominant polycystic kidney disease.