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      1. Author :
        Lorentzen, D.; Durairaj, L.; Pezzulo, A. A.; Nakano, Y.; Launspach, J.; Stoltz, D. A.; Zamba, G.; McCray, P. B., Jr.; Zabner, J.; Welsh, M. J.; Nauseef, W. M.; Banfi, B.
      2. Title :
      3. Type :
        Journal Article
      4. Year :
      5. Publication :
        Free Radic Biol Med
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      10. Research Area :
      11. Keywords :
        Xen8.1, Xen 8.1, S. aureus, IVIS, bioluminescence imaging
      12. Abstract :
        A recently discovered enzyme system produces antibacterial hypothiocyanite (OSCN(-)) in the airway lumen by oxidizing the secreted precursor thiocyanate (SCN(-)). Airway epithelial cultures have been shown to secrete SCN(-) in a CFTR-dependent manner. Thus, reduced SCN(-) availability in the airway might contribute to the pathogenesis of cystic fibrosis (CF), a disease caused by mutations in the CFTR gene and characterized by an airway host defense defect. We tested this hypothesis by analyzing the SCN(-) concentration in the nasal airway surface liquid (ASL) of CF patients and non-CF subjects and in the tracheobronchial ASL of CFTR-DeltaF508 homozygous pigs and control littermates. In the nasal ASL, the SCN(-) concentration was ~30-fold higher than in serum independent of the CFTR mutation status of the human subject. In the tracheobronchial ASL of CF pigs, the SCN(-) concentration was somewhat reduced. Among human subjects, SCN(-) concentrations in the ASL varied from person to person independent of CFTR expression, and CF patients with high SCN(-) levels had better lung function than those with low SCN(-) levels. Thus, although CFTR can contribute to SCN(-) transport, it is not indispensable for the high SCN(-) concentration in ASL. The correlation between lung function and SCN(-) concentration in CF patients may reflect a beneficial role for SCN(-).
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      14. Call Number :
        PKI @ kd.modi @ 1
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