Assessment of CFTR Dysfunction and Responsiveness to CFTR Modulators in COPD Bronchial Air-Liquid Interface Cultures. (PubMed, Int J Mol Sci) - Oct 17, 2026 - "Treatment with CFTR potentiators (VX-770, GLPG1837 and icenticaftor) resulted in modest and highly variable functional responses, with no statistically significant improvement compared with vehicle-treated controls. Collectively, these findings demonstrate abnormalities in ion transport and ciliation in the COPD airway epithelium consistent with an acquired CFTR dysfunction phenotype, but indicate that these abnormalities are not readily reversed by the tested CFTR potentiators. These results highlight differences between genetic and acquired CFTR dysfunction and underscore the need for alternative or combinatorial therapeutic strategies targeting epithelial dysfunction in COPD."
Journal • Chronic Obstructive Pulmonary Disease • Cystic Fibrosis • Genetic Disorders • Immunology • Pulmonary Disease • Respiratory Diseases • CFTR
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Andrew T Reid; Kristy S Nichol; Kurtis F Budden; Nikhil T Awatade; Peter A B Wark; Prabuddha S Pathinayake
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