Antioxidants in cystic fibrosis. Conclusions from the CF antioxidant workshop, Bethesda, Maryland, November 11-12, 2003.
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.
Cantin
André M
AM
Pulmonary Division, University of Sherbrooke, 3001, 12th Avenue North, Sherbrooke, Quebec, Canada J1H 5N4. Andre.Cantin.@USherbrooke.ca
White
Terry B
TB
Cross
Carroll E
CE
Forman
Henry Jay
HJ
Sokol
Ronald J
RJ
Borowitz
Drucy
D
D000818:Animals; D000975:Antioxidants / Q000502:physiology / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000188:drug therapy* / Q000378:metabolism; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D005978:Glutathione / Q000378:metabolism; D006801:Humans; D018384:Oxidative Stress / Q000187:drug effects; D017410:Practice Guidelines as Topic; D020545:Respiratory Mucosa / Q000187:drug effects
D000975:Antioxidants; C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D005978:Glutathione