| First Author | Werner A | Year | 2004 |
| Journal | J Lipid Res | Volume | 45 |
| Issue | 12 | Pages | 2277-86 |
| PubMed ID | 15466369 | Mgi Jnum | J:94143 |
| Mgi Id | MGI:3511382 | Doi | 10.1194/jlr.M400238-JLR200 |
| Citation | Werner A, et al. (2004) No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis. J Lipid Res 45(12):2277-86 |
| abstractText | 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, DeltaF508/DeltaF508 mice, and littermate controls. EFA metabolism was measured by (13)C stable isotope methodology in vivo. EFA status was determined by gas chromatography in tissues of cftr(-/-CAM) mice, DeltaF508/DeltaF508 mice, littermate controls, and C57Bl/6 wild types fed chow or liquid diet. After enteral administration of [(13)C]EFA, arachidonic acid (AA) and docosahexaenoic acid (DHA) were equally (13)C-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 DeltaF508/DeltaF508 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. |
