In this study, we demonstrated ADPKD cyst-lining epithelia was another source of TGF-β1 production and might act as an important TGF-β1 responsive cell type in kidney, which could enhance the production of collagen type I and fibronectin and accelerate chronic kidney disease progression under a TGF-β1 excess circumstance. This evidence concerns the gene TGFB1 and chronic kidney disease.