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- W2044477966 endingPage "a009787" @default.
- W2044477966 startingPage "a009787" @default.
- W2044477966 abstract "Our current understanding of the pathogenesis of cystic fibrosis (CF) lung disease stresses the importance of the physical and chemical properties of the airway surface liquid (ASL). In particular, the loss of cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel function in CF reduces the volume and fluidity of the ASL, thus impairing mucociliary clearance and innate antimicrobial mechanisms. Besides direct approaches to restoring mutant CFTR function, alternative therapeutic strategies may also be considered to correct the basic defect of impaired salt and water transport. Such alternative strategies are focused on the restoration of mucociliary transport by (1) reducing sodium and fluid absorption by inhibiting the ENaC channel; (2) activating alternative chloride channels; and (3) increasing airway surface hydration with osmotic agents. Therapeutic approaches directed at targets other than CFTR are attractive because they are potentially useful to all patients irrespective of their genotype. Clinical trials are underway to test the efficacy of these approaches." @default.
- W2044477966 created "2016-06-24" @default.
- W2044477966 creator A5006523632 @default.
- W2044477966 creator A5059886022 @default.
- W2044477966 date "2013-06-01" @default.
- W2044477966 modified "2023-10-17" @default.
- W2044477966 title "New Pulmonary Therapies Directed at Targets Other than CFTR" @default.
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- W2044477966 doi "https://doi.org/10.1101/cshperspect.a009787" @default.
- W2044477966 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3662351" @default.
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- W2044477966 hasPublicationYear "2013" @default.
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