Polycystin 1 and 2 (PC1 and PC2), encoded by the autosomal dominant PKD (ADPKD) genes Pkd1 and Pkd2, respectively, are targeted to cilia and this specific trafficking highly correlates with their in vivo function (Yoder et al., 2002; Barr and Sternberg, 1999; Cai et al., 2014; Pazour et al., 2002; Yoshiba et al., 2012). Here, PKD1 is linked to autosomal dominant polycystic kidney disease.