Different cytokines have diverse effects on epithelial ion channel functions and CFTR rescue (NACFC 2026) - Sep 9, 2026 - Abstract #328; "Most pwCF have at least one copy of F508del CFTR, which can be rescued with triple CFTR modulators, elexacaftor/tezacaftor/ivacaftor (ETI)...Additionally, IL-17 plus TNF- ?or IL-4 alone enhanced the activity of drug compound combinations (e.g., ELX-02 plus CC-90009 and CFTR modulators) for correcting readthrough of nonsense CFTR mutations such as G542X and W1282X [3]... Treatment with ETI plus IL-1b increased F508del CFTR activity compared to ETI alone and this enhancement was abolished by anakinra, which blocks IL-1 receptor activity [1]... Although IL-1b, IL-4, and IL-17 enhanced the function of ETIrescued F508del CFTR, with IL-1b causing the highest increase in activity, these cytokines displayed varying effects on other ion channels, i.e., ENaC and CaCC. These results suggest that distinct cytokines act on separate pathways in the airway epithelium to regulate airway surface liquid height and mucin concentration, either enhancing CFTR-mediated hydration or..."
Cystic Fibrosis • Genetic Disorders • Immunology • Infectious Disease • Respiratory Diseases • CFTR • IL17A • IL1B • IL4 • TNFA
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D. Cholon 1; S. Boyles 1; J. Minges 1; K. Okuda 2; C. Ribeiro 1; M. Gentzsch 1
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