The administration of C-176 decreased renal fibrosis as seen by a reduction of extracellular matrix deposition and a decrease of the expression of α-SMA in both Pkd1RC/RC and Pkd1fl/fl: Pkhd1-Cre kidneys compared to that in control kidneys treated with vehicle as examined by picrosirius red (Figure 5G,H) and α-SMA immunofluorescence staining analysis (Figure 5I,J). Here, PKHD1 is linked to renal fibrosis.