PKHD1 and ciliopathy: Many other genes are involved in ciliopathies, in particular, fibrocystin (PKHD1) is implicated in autosomal recessive polycystic kidney disease (ARPKD), polycystin-1 (PKD1) and polycystin-2 (PKD2) are associated to autosomal dominant polycystic kidney disease (ADPKD), and nephrocystins are correlated with nephronophthisis.