Pharmacological Improvement of CFTR Function Rescues Airway Epithelial Homeostasis and Host Defense in Children with Cystic Fibrosis

Single cell transcriptomics revealed an impaired interferon signaling and reduced expression of MHC I and II encoding genes in epithelial cells of cyctic fibrosis children at baseline, which was partially restored by elexacaftor/tezacaftor/ivacaftor.