The importance of CFTR expression for neutrophil function in patients with Cystic Fibrosis

Jundi, Bakr; Pohl, Kerstin; McElvaney,; Reeves, Emer P.

doi:10.1186/1753-6561-9-s1-a36

Cited by 3 publications

(3 citation statements)

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“…CFTR is expressed in neutrophils [ 43 – 45 ], with reduced expression observed in cells of patients with CF [ 43 ]. Neutrophils play a role in chlorination of phagocytosed bacteria, which is disrupted by CFTR channel dysfunction [ 44 ].…”

Section: The Role Of Cftr In In Nonepithelial Cellsmentioning

confidence: 99%

“…Neutrophils play a role in chlorination of phagocytosed bacteria, which is disrupted by CFTR channel dysfunction [ 44 ]. Additionally, suppression of CFTR function resulted in accumulation of cytosolic chloride in healthy neutrophils [ 43 ]. Activated neutrophils regulate CFTR distribution by mobilising it to the subcellular sites of activation, while neutrophils with the most common CFTR mutation, F508del, do not achieve this targeting and thus compromise host defence function [ 45 ].…”

Section: The Role Of Cftr In In Nonepithelial Cellsmentioning

confidence: 99%

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Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond

et al. 2022

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The cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial ion channel for transport of chloride and bicarbonate anions. Functional roles of CFTR have been identified in a broad range of cell types including epithelial, endothelial, immune, and structural cells. While CFTR has been investigated largely in the context of inborn dysfunction in cystic fibrosis (CF), recent evidence shows CFTR is also affected by acquired dysfunction in chronic obstructive pulmonary disease (COPD). In patients with COPD and smokers, CFTR impairment has been demonstrated in the upper and lower airways, sweat glands and intestines, suggesting both pulmonary and systemic defects. Cigarette smoke, a key factor in COPD development, is the major cause of acquired CFTR dysfunction. Inflammation, bacterial by-products, and reactive oxygen species can further impair CFTR expression and function. CFTR dysfunction could directly contribute to disease manifestation and progression of COPD including disturbed airway surface liquid homeostasis, airway mucus obstruction, pathogen colonisation and inflammation. Mucus plugging and neutrophilic inflammation contribute to tissue destruction, development of dysfunction at the level of the small airways and COPD progression. Acquired CFTR dysfunction in extra-pulmonary organs could add to common co-morbidities and the disease burden. This review explores how CFTR dysfunction may be acquired and its potential effects on patients with COPD, particularly those with chronic bronchitis. The development of CFTR potentiators and the probable benefits of CFTR potentiation to improve tissue homeostasis, reduce inflammation, improve host defence, and potentially reduce remodelling in the lungs will be discussed.

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Section: The Role Of Cftr In In Nonepithelial Cellsmentioning

confidence: 99%

Section: The Role Of Cftr In In Nonepithelial Cellsmentioning

confidence: 99%

Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond

et al. 2022

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“…A minor population of infiltrated neutrophils and monocytes (polarized into M2 macrophages) are channelized to phagocytose the invaded microbes. However, due to impairment in oxidative burst and lysosome-mediated pathogen digestion resulting from CF-associated dysfunctional CFTR, neutrophils and macrophages are only capable of establishing and amplifying the DAMP-mediated proinflammatory response but unable to effectively clear invaded pathogens as seen in normal individuals [ 33 , 35 , 51 , 52 ]. Once proinflammatory milieu spreads throughout the lung tissue, the resulting inflammatory response mainly through purinergic signaling further leads to complete loss of mucus clearance along with hypersecretion of mucin vesicles by the goblet cells as demonstrated in mouse models [ 17 , 22 , 46 ].…”

Section: Dysfunctional Cftr and Cystic Fibrosismentioning

confidence: 99%

Role of innate immunity and systemic inflammation in cystic fibrosis disease progression

Purushothaman

Nelson

2023

Heliyon

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Pseudomonas aeruginosa infection, but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis

Westhölter

Beckert

Straßburg

et al. 2021

Journal of Cystic Fibrosis

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The importance of CFTR expression for neutrophil function in patients with Cystic Fibrosis

Cited by 3 publications

References 0 publications

Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond

Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond

Role of innate immunity and systemic inflammation in cystic fibrosis disease progression

Pseudomonas aeruginosa infection, but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis

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