Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism

Journal of Lipid Research

Volumn 49, Issue 8, 2008, Pages 1692-1700

  Andersson, Charlotte ^a   Al Turkmani, M Rabie ^b   Savaille, Juanito E ^b   Alturkmani, Ragheed ^b   Katrangi, Waddah ^b   Cluette Brown, Joanne E ^b   Zaman, Munir M ^a   Laposata, Michael ^b   Freedman, Steven D ^a  

^a BETH ISRAEL DEACONESS MEDICAL CENTER   (United States)

^b MASSACHUSETTS GENERAL HOSPITAL   (United States)

Author keywords

Arachidonic acid; Confluence; Cystic fibrosis transmembrane conductance regulator; D6 desaturase; Docosahexaenoic acid; Essential fatty acid deficiency; Fetal bovine serum; Horse serum

Indexed keywords

ARACHIDONIC ACID; LINOLEIC ACID; TRANSMEMBRANE CONDUCTANCE REGULATOR; CFTR PROTEIN, HUMAN; FATTY ACID;

ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; CULTURE TECHNIQUE; CYSTIC FIBROSIS; EPITHELIUM CELL; FATTY ACID METABOLISM; HUMAN; HUMAN CELL; LIPID COMPOSITION; MOUSE; NONHUMAN; PRIORITY JOURNAL; RESPIRATORY EPITHELIUM; ANIMAL; BRONCHUS; CELL COUNT; CELL CULTURE; CULTURE MEDIUM; CYTOLOGY; GENETICS; METABOLISM; NUCLEIC ACID PROBE; PATHOPHYSIOLOGY; PHYSIOLOGY;

ANIMALS; ANTISENSE ELEMENTS (GENETICS); BRONCHI; CELL COUNT; CELLS, CULTURED; CULTURE MEDIA; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL CELLS; FATTY ACIDS; HUMANS; LINOLEIC ACID; MICE;

EID: 51449090990     PISSN: 00222275     EISSN: 15397262     Source Type: Journal    
DOI: 10.1194/jlr.M700388-JLR200     Document Type: Article

References (30)

1. Riordan, J. R., J. M. Rommens, B. Kerem, N. Alon, R. Rozmahel, Z. Grzelczak, J. Zielenski, S. Lok, N. Plavsic, J. L. Chou, et al. 1989. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 245: 1066-1073.
2. Kuo, P. T., N. N. Huang, and D. R. Bassett. 1962. The fatty acid composition of the serum chylomicrons and adipose tissue of children with cystic fibrosis of the pancreas. J. Pediatr. 60: 394-403.
3. Gilljam, H., B. Strandvik, A. Ellin, and L. G. Wiman. 1986. Increased mole fraction of arachidonic acid in bronchial phospholipids in patients with cystic fibrosis. Scand. J. Clin. Lab. Invest. 46: 511-518.
4. Carlstedt-Duke, J., M. Bronnegard, and B. Strandvik. 1986. Pathological regulation of arachidonic acid release in cystic fibrosis: the putative basic defect. Proc. Natl. Acad. Sci. USA. 83: 9202-9206.
5. Strandvik, B., E. Gronowitz, F. Enlund, T. Martinsson, and J. Wahlstrom. 2001. Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis. J. Pediatr. 139: 650-655.
6. Bhura-Bandali, F. N., M. Suh, S. F. Man, and M. T. Clandinin. 2000. The deltaF508 mutation in the cystic fibrosis transmembrane conductance regulator alters control of essential fatty acid utilization in epithelial cells. J. Nutr. 130: 2870-2875.
7. Ulane, M. M., J. D. Butler, A. Peri, L. Miele, R. E. Ulane, and V. S. Hubbard. 1994. Cystic fibrosis and phosphatidylcholine biosynthesis. Clin. Chim. Acta. 230: 109-116.
8. Freedman, S. D., P. G. Blanco, M. M. Zaman, J. C. Shea, M. Ollero, I. K. Hopper, D. A. Weed, A. Gelrud, M. M. Regan, M. Laposata, et al. 2004. Association of cystic fibrosis with abnormalities in fatty acid metabolism. N. Engl. J. Med. 350: 560-569.
9. -/2) mice. Proc. Natl. Acad. Sci. USA. 96: 13995-14000.
10. Zahm, J. M., D. Gaillard, F. Dupuit, J. Hinnrasky, D. Porteous, J. R. Dorin, and E. Puchelle. 1997. Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice. Am. J. Physiol. 272: C853-C859.
11. Tirouvanziam, R., S. de Bentzmann, C. Hubeau, J. Hinnrasky, J. Jacquot, B. Peault, and E. Puchelle. 2000. Inflammation and infection in naive human cystic fibrosis airway grafts. Am. J. Respir. Cell Mol. Biol. 23: 121-127.
12. -/- mice and the effect of docosahexaenoic acid. J. Appl. Physiol. 92: 2169-2176.
13. Werner, A., M. E. Bongers, M. J. Bijvelds, H. R. de Jonge, and H. J. Verkade. 2004. No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis. J. Lipid Res. 45: 2277-2286.
14. Dragomir, A., L. Hjelte, L. Hagenfeldt, and G. M. Roomans. 2004. Heparin can improve the viability of transfected cystic fibrosis cell lines in vitro. Life Sci. 75: 2203-2216.
15. Kube, D., U. Sontich, D. Fletcher, and P. B. Davis. 2001. Proinflammatory cytokine responses to P. aeruginosa infection in human airway epithelial cell lines. Am. J. Physiol. Lung Cell. Mol. Physiol. 280: L493-L502.
16. Rajan, S., G. Cacalano, R. Bryan, A. J. Ratner, C. U. Sontich, A. van Heerckeren, P. Davis, and A. Prince. 2000. Pseudomonas aeruginosa induction of apoptosis in respiratory epithelial cells: analysis of the effects of cystic fibrosis transmembrane conductance regulator dysfunction and bacterial virulence factors. Am. J. Respir. Cell Mol. Biol. 23: 304-312.
17. Cheng, S. H., S. L. Fang, J. Zabner, J. Marshall, S. Piraino, S. C. Schiavi, D. M. Jefferson, M. J. Welsh, and A. E. Smith. 1995. Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression. Am. J. Physiol. 268: L615-L624.
18. Rowe, S. M., K. Varga, A. Rab, Z. Bebok, K. Byram, Y. Li, E. J. Sorscher, and J. P. Clancy. 2007. Restoration of W1282X CFTR activity by enhanced expression. Am. J. Respir. Cell Mol. Biol. 37: 347-356.
19. Alvarez, J. G., and B. T. Storey. 1995. Differential incorporation of fatty acids into and peroxidative loss of fatty acids from phospholipids of human spermatozoa. Mol. Reprod. Dev. 42: 334-346.
20. Kuriki, K., K. Tajima, and S. Tokudome. 2006. Accelerated solvent extraction for quantitative measurement of fatty acids in plasma and erythrocytes. Lipids. 41: 605-614.
21. Flotte, T. R., S. A. Afione, R. Solow, M. L. Drumm, D. Markakis, W. B. Guggino, P. L. Zeitlin, and B. J. Carter. 1993. Expression of the cystic fibrosis transmembrane conductance regulator from a novel adeno-associated virus promoter. J. Biol. Chem. 268: 3781-3790.
22. Bailleux, A., D. Wendum, F. Audubert, A. M. Jouniaux, K. Koumanov, G. Trugnan, and J. Masliah. 2004. Cytosolic phospholipase A2-p11 interaction controls arachidonic acid release as a function of epithelial cell confluence. Biochem. J. 378: 307-315.
23. Jiang, H., A. S. Weyrich, G. A. Zimmerman, and T. M. McIntyre. 2004. Endothelial cell confluence regulates cyclooxygenase-2 and prostaglandin E2 production that modulate motility. J. Biol. Chem. 279: 55905-55913.
24. Marcel, Y. L., K. Christiansen, and R. T. Holman. 1968. The preferred metabolic pathway from linoleic acid to arachidonic acid in vitro. Biochim. Biophys. Acta. 164: 25-34.
25. Bernert, J. T., Jr., and H. Sprecher. 1975. Studies to determine the role rates of chain elongation and desaturation play in regulating the unsaturated fatty acid composition of rat liver lipids. Biochim. Biophys. Acta. 398: 354-363.
26. Kokoglu, E., Y. Tuter, K. S. Sandikci, Z. Yazici, E. Z. Ulakoglu, H. Sonmez, and E. Ozyurt. 1998. Prostaglandin E2 levels in human brain tumor tissues and arachidonic acid levels in the plasma membrane of human brain tumors. Cancer Lett. 132: 17-21.
27. -/-) knockout mice. Metabolism. 55: 1192-1200.
28. Lai, J. Y., and M. R. Pittelkow. 2004. Culture confluence regulates gene expression of normal human keratinocytes. Wound Repair Regen. 12: 613-617.
29. Xu, F., and Z. J. Zhao. 2001. Cell density regulates tyrosine phosphorylation and localization of focal adhesion kinase. Exp. Cell Res. 262: 49-58.
30. Sood, R., C. Bear, W. Auerbach, E. Reyes, T. Jensen, N. Kartner, J. R. Riordan, and M. Buchwald. 1992. Regulation of CFTR expression and function during differentiation of intestinal epithelial cells. EMBO J. 11: 2487-2494.