In this study, using cultured MLE12 cells and alveolar epithelial cell-specific FUT8 conditional knockout (CKO) mouse models, we show that CF mediates the modification of TGF-βR I which is a critical module in the TGF-β pathway and regulates cell senescence induced by bleomycin (BLM) in vivo and in vitro. Here, TGFB1 is linked to cystic fibrosis.