 Autosomal dominant polycystic kidney disease ADPKD is a life-threatening monogenic disease with a prevalence of 1 in 400-1000 livebirths. ADPKD is caused by mutations to polycystic kidney disease 1 gene PKD1 approximately 85 of cases or polycystic kidney disease 2 gene PKD2 the remaining 15 gene, encoding polycystin-1 PC1 and polycystin-2 PC2, respectively. PC1 is a putative cell-surface, receptor-like protein with yet to-be-identified ligands, and PC2 a channel protein with a high conductance to Ca2. 