CFTR dysfunction leads to defective bacterial eradication on cystic fibrosis airways

TL;DR

This paper explores how CFTR dysfunction in cystic fibrosis leads to bacterial infections in the lungs, contributing to disease progression.

Contribution

The paper critically reviews current hypotheses linking CFTR dysfunction to bacterial infection in cystic fibrosis airways.

Findings

CFTR dysfunction leads to mucus accumulation and bacterial infection in CF airways.

Loss of CFTR function disrupts ion transport and epithelial function, promoting lung disease.

Understanding CFTR's role may guide new treatments for cystic fibrosis.

Abstract

Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel by genetic mutations causes the inherited disease cystic fibrosis (CF). CF lung disease that involves multiple disorders of epithelial function likely results from loss of CFTR function as an anion channel conducting chloride and bicarbonate ions and its function as a cellular regulator modulating the activity of membrane and cytosol proteins. In the absence of CFTR activity, abundant mucus accumulation, bacterial infection and inflammation characterize CF airways, in which inflammation-associated tissue remodeling and damage gradually destroys the lung. Deciphering the link between CFTR dysfunction and bacterial infection in CF airways may reveal the pathogenesis of CF lung disease and guide the development of new treatments. Research efforts towards this goal, including high salt, low volume, airway surface liquid acidosis and abnormal mucus hypotheses are critically reviewed.

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