We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Cystic fibrosis in the era of CFTR modulators: did the neutrophil slip through the cracks?
- Authors
Witko-Sarsat, Véronique; Burgel, Pierre-Régis
- Abstract
Cystic fibrosis (CF) is a genetic disease caused by mutations in the CFTR gene, resulting in abnormalities in the transport of chloride and bicarbonate anions in the airway epithelium. This leads to mucus dehydration and plugging, as well as persistent bacterial infection despite the presence of neutrophils. Recent studies have shown that CFTR is present in neutrophils and plays a role in their function, including antimicrobial action and cell death pathways. The therapeutic application of CFTR modulators has improved clinical outcomes in CF patients, but little is known about the effects of these modulators on neutrophil function. A recent study analyzed the genetic program of neutrophils from CF patients treated with CFTR modulators and found that the dysfunction of CF neutrophils persists despite treatment. However, further research is needed to understand the effects of CFTR modulators on neutrophil abnormalities and to develop targeted therapies for CF.
- Subjects
CYSTIC fibrosis; CYSTIC fibrosis transmembrane conductance regulator; NEUTROPHILS
- Publication
Journal of Leukocyte Biology, 2024, Vol 115, Issue 3, p417
- ISSN
0741-5400
- Publication type
Article
- DOI
10.1093/jleuko/qiad164