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Prostaglandins Leukot Essent Fatty Acids. 2010 Sep;83(3):121-9. doi: 10.1016/j.plefa.2010.07.002. Epub 2010 Jul 31.

Fatty acid metabolism in cystic fibrosis.

Author information

1
Department of Biosciences and Nutrition, Unit of Public Health Nutrition, NOVUM, Karolinska Institutet, Hälsovägen 7, 14157 Huddinge, Stockholm, Sweden. Birgitta.Strandvik@ki.se

Abstract

Despite identification twenty years ago of the gene responsible for cystic fibrosis transmembrane conductance regulator (CFTR), the protein defective in cystic fibrosis (CF), research of this monogenetic disease has not provided an explanation for the divergent symptoms, and a treatment breakthrough is still awaited. This review discusses different aspects of disturbances in lipid metabolism seen in CF. These include increased release of arachidonic acid (AA) from cell membrane phospholipids and a low status of linoleic and docosahexaenoic acids. Recent research has explored more complicated lipid associations. Disturbances in annexins and ceramides might act in concert to explain the impact on inflammation and AA release. The connections to CFTR and between the disturbances in essential fatty acid metabolism are reviewed. The metabolic interactions, some of which might be compensating, possibly explain the difficulties in understanding the fatty acid disturbances in relation to different symptoms and their relation to the defective CFTR.

PMID:
20673710
DOI:
10.1016/j.plefa.2010.07.002
[Indexed for MEDLINE]

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