Influence of highly effective modulator therapy on the sputum proteome in cystic fibrosis

Cystic fibrosis (CF) is an inherited, multi-system disease caused by dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, a ubiquitous ion channel important for epithelial hydration [1]. A direct consequence of this dysfunction is impaired mucociliary clearance, chronic airway infection and a persistent neutrophilic inflammatory response that results in progressive loss of lung function, development of respiratory failure and premature death [2]. Partial restoration of CFTR function is now possible for most people with CF (PwCF) through mutation specific CFTR modulators.

Source: Journal of Cystic Fibrosis - November 9, 2023 Category: Respiratory Medicine Authors: Rosemary E Maher, Peter J Barry, Edward Emmott, Andrew M Jones, Lijing Lin, Paul S McNamara, Jaclyn A Smith, Robert W Lord Tags: Original Article Source Type: research