Journal
FREE RADICAL BIOLOGY AND MEDICINE
Volume 42, Issue 1, Pages 15-31Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.freeradbiomed.2006.09.022
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Funding
- NIEHS NIH HHS [R01 ES005511, R01 ES005511-18A2] Funding Source: Medline
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Although great strides are being made in the care of individuals with cystic fibrosis (CF), this condition remains the most common fatal hereditary disease in North America. Numerous links exist between progression of CF lung disease and oxidative stress. The defect in CF is the loss of function of the transmembrane conductance regulator (CFTR) protein; recent evidence that CFTR expression and function are modulated by oxidative stress suggests that the loss may result in a poor adaptive response to oxidants. Pancreatic insufficiency in CF also increases susceptibility to deficiencies in lipophilic antioxidants. Finally the airway infection and inflammatory processes in the CF lung are potential sources of oxidants that can affect normal airway physiology and contribute to the mechanisms causing characteristic changes associated with bronchiectasis and loss of lung function. These multiple abnormalities in the oxidant/antioxidant balance raise several possibilities for therapeutic interventions that must be carefully assessed. (c) 2006 Elsevier Inc. All rights reserved.
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