Although murine models of CF do not recapitulate the clinical manifestations of CF lung disease, CF mice manifest an inflammatory lung phenotype coupled to hyper-responsiveness to bacteria or microbial products and are widely used to test candidate drugs and their mechanism of action.47 In addition, we used autophagy-defective Becn1+/− mice as well as CftrF508del/F508del mice in a background of BECN1 haploinsufficiency (CftrF508del/F508del/Becn1+/− mice). Here, BECN1 is linked to cystic fibrosis.