期刊
FREE RADICAL BIOLOGY AND MEDICINE
卷 50, 期 9, 页码 1144-1150出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.freeradbiomed.2011.02.013
关键词
Thiocyanate; Dual oxidase; Lactoperoxidase; Cystic fibrosis; Airway surface liquid; Free radicals
资金
- Cystic Fibrosis Foundation Therapeutics [R458-CR02, BANF107A0]
- NHLBI [HL090830, HL91842, HL51670]
- NIH [K23 HL075402]
- Cystic Fibrosis Foundation [R458-CR02, ENGLH9850]
- NIDDK [DK54759]
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-Delta F508 homozygous pigs and control littermates. In the nasal ASL, the SCN- concentration was similar to 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-. (C) 2011 Elsevier Inc. All rights reserved.
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