We also found that treatment with C-176 induced cyst-lining epithelial cell death in kidneys from Pkd1RC/RC and Pkd1fl/fl: Pkhd1-Cre mice compared to that in kidneys from age-matched control mice treated with vehicle as analyzed by the terminal-deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) assay (Figure 6A,B). Here, PKHD1 is linked to cyst.