Gene expression responses of CF airway epithelial cells exposed to elexacaftor/tezacaftor/ivacaftor suggest benefits beyond improved CFTR channel function
Thomas H. Hampton, Roxanna Barnaby, Carolyn Roche, Amanda Nymon, Kiyoshi F. Fukutani, Todd A. MacKenzie, L. Charpentier, Bruce A. Stanton
2024American Journal of Physiology-Lung Cellular and Molecular Physiology18 citationsDOIOpen Access PDF
Abstract
Gene expression responses by CF AECs exposed to ETI suggest that in addition to improving CFTR channel function, ETI is likely to enhance resistance to bacterial infection by increasing levels of beta-defensin 1 (hBD-1). ETI may also reduce lung damage by suppressing MMP10 and MMP12 and reduce airway inflammation by repressing proinflammatory cytokine secretion by CF AECs.
Topics & Concepts
IvacaftorHMOX1Cystic fibrosisGene expressionBiologyTranscriptomeInflammationGeneCell biologyImmunologyMolecular biologyCancer researchCystic fibrosis transmembrane conductance regulatorHeme oxygenaseHemeGeneticsBiochemistryEnzymeCystic Fibrosis Research AdvancesPediatric health and respiratory diseasesNeonatal Respiratory Health Research