PKHD1 and autosomal recessive polycystic kidney disease: ADPKD, ARPKD and NPHP are caused by mutations in the Pkd1/Pkd2, Pkhd1 and Nphp (Grantham et al., 1989, 2011; Wilson et al., 1999; Schwiebert, 2001; Rangan et al., 2005; Wang et al., 2005; Harris and Torres, 2009; Xu et al., 2009; Aguiari et al., 2013; Chang and Ong, 2013; Kelsey, 2013) genes respectively, which encode the proteins, polycystin (PC)-1/PC-2, fibrocystin and nephrocystin (Sweeney and Avner, 2006; Harris and Torres, 2009).