J. Lipid Res.
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Originally published In Press as doi:10.1194/jlr.M400238-JLR200 on October 1, 2004

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Journal of Lipid Research, Vol. 45, 2277-2286, December 2004
Copyright © 2004 by American Society for Biochemistry and Molecular Biology

No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis

Anniek Werner*, Marloes E. J. Bongers*, Marcel J. Bijvelds{dagger}, Hugo R. de Jonge{dagger} and Henkjan J. Verkade1,*

* Department of Pediatrics, Center for Liver, Digestive and Metabolic Diseases, Academic Hospital Groningen, The Netherlands
{dagger} Department of Biochemistry, Erasmus Medical Center, Rotterdam, The Netherlands

1 To whom correspondence should be addressed. e-mail: h.j.verkade{at}med.rug.nl

A deficiency of essential fatty acids (EFA) is frequently described in cystic fibrosis (CF), but whether this is a primary consequence of altered EFA metabolism or a secondary phenomenon is unclear. It was suggested that defective long-chain polyunsaturated fatty acid (LCPUFA) synthesis contributes to the CF phenotype. To establish whether cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction affects LCPUFA synthesis, we quantified EFA metabolism in cftr–/–CAM and cftr+/+CAM mice. Effects of intestinal phenotype, diet, age, and genetic background on EFA status were evaluated in cftr–/–CAM mice, {Delta}F508/{Delta}F508 mice, and littermate controls. EFA metabolism was measured by 13C stable isotope methodology in vivo. EFA status was determined by gas chromatography in tissues of cftr–/–CAM mice, {Delta}F508/{Delta}F508 mice, littermate controls, and C57Bl/6 wild types fed chow or liquid diet. After enteral administration of [13C]EFA, arachidonic acid (AA) and docosahexaenoic acid (DHA) were equally 13C-enriched in cftr–/–CAM and cftr+/+CAM mice, indicating similar EFA elongation/desaturation rates. LA, ALA, AA, and DHA concentrations were equal in pancreas, lung, and jejunum of chow-fed cftr–/–CAM and {Delta}F508/{Delta}F508 mice and controls. LCPUFA levels were also equal in liquid diet-weaned cftr–/–CAM mice and littermate controls, but consistently higher than in age- and diet-matched C57Bl/6 wild types. We conclude that cftr–/–CAM mice adequately absorb and metabolize EFA, indicating that CFTR dysfunction does not impair LCPUFA synthesis.

A membrane EFA imbalance is not inextricably linked to the CF genotype. EFA status in murine CF models is strongly determined by genetic background.

Abbreviations: AA, arachidonic acid, 20:4n-6; ALA, {alpha}-linolenic acid, 18:3n-3; CF, cystic fibrosis; CFTR, cystic fibrosis transmembrane conductance regulator; DHA, docosahexaenoic acid, 22:6n-3; EFA, essential fatty acid; KHB, Krebs-Henseleit buffer; LA, linoleic acid, 18:2n-6; LCPUFA, long-chain polyunsaturated fatty acid; PL, phospholipids

Supplementary key words arachidonic acid • cystic fibrosis transmembrane conductance regulator • docosahexaenoic acid • essential fatty acid deficiency • {alpha}-linolenic acid • linoleic acid • modifier genes


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