Using these approaches, good models of progressive disease, modeling human ADPKD but with more rapid progression, have been developed (including Pkd1L3/L3, Pkd1RC/RC, and Pkd2nf3/nf3; Table 1).66,68, –70 For ARPKD, complete loss of Pkhd1 in the mouse results in only mild, late-onset tubule dilatation, although the liver phenotype mirrors the human disease (Supplemental Table 1). The gene discussed is PKHD1; the disease is autosomal dominant polycystic kidney disease.