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Papers In Press, published online ahead of print October 1, 2004
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Department of Pediatric and Gastroenterology, Academic Hospital Groningen, Groningen 9700 RB
Corresponding Author: a.werner{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 CF-phenotype. To establish whether 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,
Revised on September 16, 2004
Accepted on September 20, 2004
No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis
F508/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, F508/F508 mice, littermate controls and C57Bl/6 wildtypes, 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 F508/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 wildtypes. 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 CF-genotype. EFA-status in murine CF-models is strongly determined by genetic background.
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