In this study, we investigate the role and mechanism of STING in ADPKD by focusing on whether and how STING regulates immune responses and macrophage recruitment as well as cystic epithelial cell apoptosis and renal fibrosis in Pkd1 mutant mouse kidneys, and testing whether targeting STING with a specific inhibitor delays cyst growth in two Pkd1 mutant mouse models. Here, PKD1 is linked to cyst.