Year: 2026
Type: article
Abstract: BACKGROUND: Cystic fibrosis (CF) is a hereditary disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The major causes of morbidity and mortality in CF are related to lung disease, involving neutrophil-dominated lung inflammation. SUMMARY: Whether the altered inflammatory response of neutrophils in patients with CF is an intrinsic defect due to a lack of CFTR protein expression, or alternatively, exacerbated by chronic exposure to infection and inflammation, is extensively debated. Fuelling this dispute are conflicting past studies on CFTR expression by neutrophils, with opposing results described at both the gene and protein level. This is pertinent in the era of CFTR modulator therapies, with clinicians and scientists exploring the impact of different CFTR mutation classes and CFTR modulators on neutrophil function. KEY MESSAGES: The focus of this article was to uncover the cause for the described disparity of data on neutrophil CFTR expression, by investigating methods utilised for CFTR detection and drawing consensus on the optimal protocol for identifying CFTR protein in neutrophils.
Source: Journal of Innate Immunity
DOI: 10.1159/000551637
Institution: Royal College of Surgeons in Ireland
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,590
OpenAlex ID: W7139942010
FWCI:
Cites:
Cited by:
Related to: