CFTR‐dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy
Sign Up to like & getrecommendations! Published in 2017 at "Pediatric Pulmonology"
DOI: 10.1002/ppul.23712
Abstract: The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) potentiator ivacaftor (Kalydeco®) improves clinical outcome in G551D cystic fibrosis (CF) patients. Here, we have investigated whether ivacaftor has a clinical impact on non‐G551D gating mutations and function… read more here.
Keywords: ivacaftor; cystic fibrosis; fibrosis; cftr dependent ... See more keywords
CAGE sequencing reveals CFTR-dependent dysregulation of type I IFN signaling in activated cystic fibrosis macrophages
Sign Up to like & getrecommendations! Published in 2023 at "Science Advances"
DOI: 10.1126/sciadv.adg5128
Abstract: An intense, nonresolving airway inflammatory response leads to destructive lung disease in cystic fibrosis (CF). Dysregulation of macrophage immune function may be a key facet governing the progression of CF lung disease, but the underlying… read more here.
Keywords: ifn signaling; type ifn; dysregulation; cftr dependent ... See more keywords
IL-13–programmed airway tuft cells produce PGE2, which promotes CFTR-dependent mucociliary function
Sign Up to like & getrecommendations! Published in 2022 at "JCI Insight"
DOI: 10.1172/jci.insight.159832
Abstract: Chronic type 2 (T2) inflammatory diseases of the respiratory tract are characterized by mucus overproduction and disordered mucociliary function, which are largely attributed to the effects of IL-13 on common epithelial cell types (mucus secretory… read more here.
Keywords: airway tuft; mucociliary function; tuft cells; cftr dependent ... See more keywords