For example, autosomal dominant and recessive PKD (ADPKD and ARPKD), and nephronopthisis, are ciliopathies caused by mutations in genes encoding the ciliary proteins polycystin‐1 (Consortium 1995; Hughes et al. 1995; Yoder et al. 2002) (PC‐1, ADPKD), PC‐2 (Mochizuki et al. 1996; Yoder et al. 2002; Boletta and Germino 2003) (ADPKD), fibrocystin (Zerres et al. 1994; Onuchic et al. 2002; Ward et al. 2002, 2003) (ARPKD), and the nephrocystins (nephronophthisis) (Jauregui 2007; Hurd and Hildebrandt 2010). This evidence concerns the gene PKHD1 and autosomal recessive polycystic kidney disease.