Cystic fibrosis2 of 2
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In CF, the absence of CFTR protein/function leads to dehydration of the ASL and excess secretion of viscous mucus.1-4
In CF, there is reduced quantity and/or function of CFTR in the apical membrane.1
As ENaC is not down-regulated by CFTR, there is a hyperabsorption of Na+ into epithelia cells. This is followed by absorption of water and Cl– and via non-CFTR channels.1-4
As a consequence, there is a worsening degree of airway surface dehydration and decreased mucociliary clearance. Bacteria and mucus plaques accumulate leading to recurrent chronic endobronchial infections (e.g. Pseudomonas aeruginosa), bronchiectasis, and ultimately end-stage lung disease.5