Many ADPKD mouse models have been generated for Pkd1 and Pkd2, but because heterozygotes of fully inactivating alleles (modeling the human disease) only develop a few cysts and homozygotes are not viable71,79 (also true for rat Pkd180), other approaches are required to model the progressive human disease. Here, PKD2 is linked to autosomal dominant polycystic kidney disease.