Neutrophil dysfunction in cystic fibrosis

Cystic fibrosis (CF), a genetic disease caused by a mutated cystic fibrosis transmembrane conductance regulator (CFTR) gene that results in a dysfunctional or absent epithelial chloride channel, is characterized by maladaptive neutrophilic inflammation. Clinically, individuals with CF have chronic airway infections, with thick neutrophil-laden mucus, which leads to a progressive decline in lung function, ultimately resulting in early death from respiratory failure. As neutrophils are key drivers of the hyper-inflammatory state associated with CF, there remains considerable interest in understanding how CF neutrophils function in comparison to non-CF neutrophils.

Source: Journal of Cystic Fibrosis - February 12, 2021 Category: Respiratory Medicine Authors: Lael M. Yonker, Anika Marand, Sinan Muldur, Alex Hopke, Hui Min Leung, Denis De La Flor, Grace Park, Hanna Pinsky, Lauren B. Guthrie, Guillermo J. Tearney, Daniel Irimia, Bryan P. Hurley Tags: Original Article Source Type: research