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Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions
Journal article   Peer reviewed

Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions

Daniel Lorentzen, Lakshmi Durairaj, Alejandro A Pezzulo, Yoko Nakano, Janice Launspach, David A Stoltz, Gideon Zamba, Paul B McCray, Joseph Zabner, Michael J Welsh, …
Free radical biology & medicine, Vol.50(9), pp.1144-1150
05/01/2011
DOI: 10.1016/j.freeradbiomed.2011.02.013
PMCID: PMC3070840
PMID: 21334431

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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-ΔF508 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−.
Airway surface liquid Cystic fibrosis Free radicals Lactoperoxidase Dual oxidase Thiocyanate

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