In the case of loss of Pkd1 or Pkd2 in the mouse, development of ADPKD depends on intact cilia; inappropriate signalling mediated by cilia upon disruption of PKD1/2 leading to cysts can be partially rescued by ablating cilia via Ift20 and/or Kif3a knockout 192. Here, PKD1 is linked to autosomal dominant polycystic kidney disease.