Autosomal dominant polycystic kidney disease (ADPKD) is due to germ-line variants, predominantly in either PKD1 (85%) or PKD2 (15%), encoding the polytopic integral membrane, polycystin-1, and the calcium transient receptor channel, polycystin-2, respectively [1,2,3]. Here, PKD1 is linked to autosomal dominant polycystic kidney disease.