Dysfunctional cystin (Hou et al., 2002) and polaris, located in the ciliary basal body and axoneme (Yoder et al., 2002), and the ciliary channel/receptor complex proteins polycystins 1 (PC1) and 2 (PC2), which cause ADPKD, generate ciliopathies (Murcia et al., 2000; Hou et al., 2002; Pazour et al., 2002; Yoder et al., 2002). Here, PKD2 is linked to autosomal dominant polycystic kidney disease.