As with other disease preclinical models, the ADPKD mouse models do not perfectly recapitulate the human disease; attempted full Pkd1 and Pkd2 null mouse is embryonic lethal, which has led to models such as the ones used here that are expressed in the kidney only or as an inducible model to closer mimic adult onset ADPKD (Traykova-Brauch et al. 2008; Williams et al. 2014). Here, PKD2 is linked to autosomal dominant polycystic kidney disease.