Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype

Chronic airway inflammation underpins the progression of CF lung disease and is consequently a major focus of CF research and an important therapeutic target. There is ample evidence of dysregulated inflammatory pathways in immune cells derived from people with CF and the observation of functional CFTR expression in non-epithelial cells has uncovered a direct role for the basic defect in immune cell dysfunction in CF, particularly in monocytes and macrophages [1 –3]. Rollout of disease-modifying CFTR modulators to the vast majority of people with CF may now place further emphasis on animal models to provide a means of investigating baseline differences in uncorrected, CFTR-deficient immune cells with the aim of developing novel strategies to combat chroni c inflammation alongside modulator therapies and the standard treatment regimen [4].

Source: Journal of Cystic Fibrosis - November 16, 2021 Category: Respiratory Medicine Authors: Jonathan L Gillan, Gareth R Hardisty, Donald J Davidson, Robert D Gray Source Type: research