| Title | Cystic Fibrosis and Nutrition: Linking Phospholipids and Essential Fatty acids with Thiol Metabolism. | | Author(s) | Innis SM, Davidson AG | | Institution | Nutrition Research Program, Child and Family Research Institute, and Cystic Fibrosis Clinic, Department of Paediatrics, Faculty of Medicine, University of British Columbia, Vancouver, Canada V5Z H4H; email: sinnis@nutrition.ubc.ca. | | Source | Annu Rev Nutr 2008 May 22. | | Abstract | Cystic fibrosis (CF) is the mostcommonlethal 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 Sadenosylmethionine, 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. Expected final online publication date for the Annual Review of Nutrition Volume 28 is July 17, 2008. Please see http://www.annualreviews.org/catalog/pubdates.aspx for revised estimates. | | Language | ENG | | Pub Type(s) | JOURNAL ARTICLE
| | PubMed ID | 18498235 |
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