Cystic Fibrosis and Nutrition: Linking Phospholipids and Essential Fatty Acids with Thiol Metabolism

Cystic fibrosis (CF) is the most common lethal inherited disorder among Caucasians and results from mutation in the gene encoding the CF transmembrane conductance regulator. In addition to its multisystem clinical effects, the disease is characterized by increased proinflammatory mediators and oxida...

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Bibliographic Details
Published in:Annual review of nutrition 2008-01, Vol.28 (1), p.55-72
Main Authors: Innis, Sheila M, Davidson, A. George F
Format: Article
Language:English
Subjects:
antioxidants
Biological and medical sciences
cystic fibrosis
Cystic Fibrosis - genetics
Cystic Fibrosis - metabolism
Cystic Fibrosis - pathology
cytokines
diet therapy
dietary supplements
docosahexaenoic acid
Epithelial Cells - metabolism
essential fatty acids
Fatty Acids, Essential - metabolism
Gastroenterology. Liver. Pancreas. Abdomen
Gene Expression
glutathione
Humans
inflammation
lipid metabolism
Lipid Metabolism - physiology
literature reviews
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Medical sciences
Mutation
omega-3 fatty acids
omega-6 fatty acids
Other diseases. Semiology
oxidative stress
phosphatidylcholines
phospholipids
Phospholipids - metabolism
receptors
thiols
transmembrane proteins
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Summary:Cystic fibrosis (CF) is the most common lethal inherited disorder among Caucasians and results from mutation in the gene encoding the CF transmembrane conductance regulator. In addition to its multisystem clinical effects, the disease is characterized by increased proinflammatory mediators and oxidant stress, and systemic redox imbalance with reduced glutathione (GSH), together with alterations in circulating and tissue (n-6) and (n-3) fatty acids, particularly a decrease in docosahexaenoic acid. The metabolism of phospholipids and fatty acids is closely related to GSH through the methionine-homocysteine cycle, in which choline via betaine provides methyl groups to regenerate S-adenosylmethionine, important in generating phosphatidylcholine and amino acid precursors for GSH. Current research focuses both on fatty acid supplementations to normalize altered (n-6) to (n-3) fatty acid balance and decrease generation of (n-6) fatty acid-derived inflammatory mediators, and strategies to improve oxidant defenses and redox balance. However, further research is needed before such strategies can be included in clinical care of individuals with CF.
ISSN:0199-9885
1545-4312
DOI:10.1146/annurev.nutr.27.061406.093625