CFTR and cystic fibrosis: Although arising in the absence of any defects in CFTR, NCFBE shares many features with CF including impaired mucociliary clearance, chronic bacterial infection, frequent viral exacerbations, chronic inflammation (typically involving accumulation of activated neutrophils) and progressively declining lung function.64, 65, 66, 67 Despite these common features, our studies have failed to demonstrate any clear evidence of ER stress and UPR activation in NCFBE.