PKD1 and autosomal dominant polycystic kidney disease: PKD is the most common inherited renal disease (≈1:1000) [259,260] and its most prevalent form, Autosomal Dominant PKD (ADPKD) is caused by mutations in one of two primary cilium-associated membrane proteins, polycystin 1 (PC1 or PKD1, Pkd1 gene product, 85%) and polycystin 2 (PC2 or PKD2, Pkd2 gene product, 15%) [175,261].