DHA does not protect ELOVL4 transgenic mice from retinal degeneration

Molecular Vision

Volumn 15, Issue , 2009, Pages 1185-1193

  Li, Feng ^a,b   Marchette, Lea D ^a,b   Brush, Richard S ^a,b   Elliott, Michael H ^a,b   Le, Yun Zheng ^a   Henry, Kimberly A ^a,b   Anderson, Ashley G ^a,b   Zhao, Chao ^c   Sun, Xufang ^c   Zhang, Kang ^c   Anderson, Robert E ^a,b  

^a UNIVERSITY OF OKLAHOMA COLLEGE OF MEDICINE   (United States)

^b UNIVERSITY OF OKLAHOMA HEALTH SCIENCES CENTER   (United States)

^c UNIVERSITY OF CALIFORNIA SAN DIEGO   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DOCOSAHEXAENOIC ACID; ELONGATION OF VERY CHAIN FATTY ACID 4; GENE PRODUCT; POLYUNSATURATED FATTY ACID; PROTEIN FAT 1; RHODOPSIN; SAFFLOWER OIL; UNCLASSIFIED DRUG; VERY LONG CHAIN FATTY ACID;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL VIABILITY; CONTROLLED STUDY; DIET SUPPLEMENTATION; ELECTRORETINOGRAPHY; FATTY ACID ANALYSIS; FATTY ACID BLOOD LEVEL; GENOTYPE; LIVER; MOUSE; MUTANT; NONHUMAN; PHOTORECEPTOR CELL; PRIORITY JOURNAL; RETINA; RETINA ROD OUTER SEGMENT; SAFFLOWER; STARGARDT DISEASE; TRANSGENIC MOUSE;

ANIMALS; CAENORHABDITIS ELEGANS PROTEINS; DOCOSAHEXAENOIC ACIDS; ELECTRORETINOGRAPHY; EYE PROTEINS; FATTY ACID DESATURASES; FATTY ACIDS; FEMALE; GENE EXPRESSION; HUMANS; IMMUNOHISTOCHEMISTRY; LIVER; MALE; MEMBRANE PROTEINS; MICE; MICE, TRANSGENIC; RETINA; RETINAL DEGENERATION; RHODOPSIN;

EID: 67650077520     PISSN: None     EISSN: 10900535     Source Type: Journal    
DOI: None     Document Type: Article

References (43)

1. Edwards AO, Donoso LA, Ritter R III. A novel gene for autosomal dominant Stargardt-like macular dystrophy with homology to the SUR4 protein family. Invest Ophthalmol Vis Sci 2001; 42:2652-63.
2. Zhang K, Kniazeva M, Han M, Li W, Yu Z, Yang Z, Li Y, Metzker ML, Allikmets R, Zack DJ, Kakuk LE, Lagali PS, Wong PW, MacDonald IM, Sieving PA, Figueroa DJ, Austin CP, Gould RJ, Ayyagari R, Petrukhin K. A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy. Nat Genet 2001; 27:89-93.
3. Mandal MN, Ambasudhan R, Wong PW, Gage PJ, Sieving PA, Ayyagari R. Characterization of mouse orthologue of ELOVL4: genomic organization and spatial and temporal expression. Genomics 2004; 83:626-35.
4. Lagali PS, Liu J, Ambasudhan R, Kakuk LE, Bernstein SL, Seigel GM, Wong PW, Ayyagari R. Evolutionarily conserved ELOVL4 gene expression in the vertebrate retina. Invest Ophthalmol Vis Sci 2003; 44:2841-50.
5. Zhang XM, Yang Z, Karan G, Hashimoto T, Baehr W, Yang XJ, Zhang K. Elovl4 mRNA distribution in the developing mouse retina and phylogenetic conservation of Elovl4 genes. Mol Vis 2003; 9:301-7.
6. Karan G, Lillo C, Yang Z, Cameron DJ, Locke KG, Zhao Y, Thirumalaichary S, Li C, Birch DG, Vollmer-Snarr HR, Williams DS, Zhang K. Lipofuscin accumulation, abnormal electrophysiology, and photoreceptor degeneration in mutant ELOVL4 transgenic mice: a model for macular degeneration. Proc Natl Acad Sci USA 2005; 102:4164-9.
7. Jakobsson A, Westerberg R, Jacobsson A. Fatty acid elongases in mammals: their regulation and roles in metabolism. Prog Lipid Res 2006; 45:237-49.
8. Tinoco J. Dietary requirements and functions of alpha-linolenic acid in animals. Prog Lipid Res 1982; 21:1-45.
9. Fliesler SJ, Anderson RE. Chemistry and metabolism of lipids in the vertebrate retina. Prog Lipid Res 1983; 22:79-131.
10. Anderson RE, Penn JS. Environmental light and heredity are associated with adaptive changes in retinal DHA levels that affect retinal function. Lipids 2004; 39:1121-4.
11. Benolken RM, Anderson RE, Wheeler TG. Membrane fatty acids associated with the electrical response in visual excitation. Science 1973; 182:1253-4.
12. Birch DG, Birch EE, Hoffman DR, Uauy RD. Retinal development in very-low-birth-weight infants fed diets differing in omega-3 fatty acids. Invest Ophthalmol Vis Sci 1992; 33:2365-76.
13. Birch EE, Birch DG, Hoffman DR, Uauy R. Dietary essential fatty acid supply and visual acuity development. Invest Ophthalmol Vis Sci 1992; 33:3242-53.
14. Bush RA, Reme CE, Malnoe A. Light damage in the rat retina: the effect of dietary deprivation of N-3 fatty acids on acute structural alterations. Exp Eye Res 1991; 53:741-52.
15. Jeffrey BG, Mitchell DC, Gibson RA, Neuringer M. n-3 fatty acid deficiency alters recovery of the rod photoresponse in rhesus monkeys. Invest Ophthalmol Vis Sci 2002; 43:2806-14.
16. Mitchell DC, Niu SL, Litman BJ. Enhancement of G protein-coupled signaling by DHA phospholipids. Lipids 2003; 38:437-43.
17. Neuringer M, Connor WE, Van Petten C, Barstad L. Dietary omega-3 fatty acid deficiency and visual loss in infant rhesus monkeys. J Clin Invest 1984; 73:272-6.
18. Neuringer M, Connor WE, Lin DS, Barstad L, Luck S. Biochemical and functional effects of prenatal and postnatal omega 3 fatty acid deficiency on retina and brain in rhesus monkeys. Proc Natl Acad Sci USA 1986; 83:4021-5.
19. Niu SL, Mitchell DC, Lim SY, Wen ZM, Kim HY, Salem N Jr, Litman BJ. Reduced G protein-coupled signaling efficiency in retinal rod outer segments in response to n-3 fatty acid deficiency. J Biol Chem 2004; 279:31098-104.
20. Uauy RD, Birch DG, Birch EE, Tyson JE, Hoffman DR. Effect of dietary omega-3 fatty acids on retinal function of very-low-birth-weight neonates. Pediatr Res 1990; 28:485-92.
21. Wheeler TG, Benolken RM, Anderson RE. Visual membranes: specificity of fatty acid precursors for the electrical response to illumination. Science 1975; 188:1312-4.
22. Hubbard AF, Askew EW, Singh N, Leppert M, Bernstein PS. Association of adipose and red blood cell lipids with severity of dominant Stargardt macular dystrophy (STGD3) secondary to an ELOVL4 mutation. Arch Ophthalmol 2006; 124:257-63.
23. MacDonald IM, Hébert M, Yau RJ, Flynn S, Jumpsen J, Suh M, Clandinin MT. Effect of docosahexaenoic acid supplementation on retinal function in a patient with autosomal dominant Stargardt-like retinal dystrophy. Br J Ophthalmol 2004; 88:305-6.
24. Sauve Y, Mema SC, Kuny S, Gaillard F, Zhang K, MacDonald IM, Clindin T. Dietary docosahexaenoic acid (DHA) supplementation in a mouse model of Stargardt-like dystrophy and wild type mice delays age-related changes in retina function. ARVO Annual Meeting; 2009 May 3-7; Fort Lauderdale (FL).
25. Stinson AM, Wiegand RD, Anderson RE. Recycling of docosahexaenoic acid in rat retinas during n-3 fatty acid deficiency. J Lipid Res 1991; 32:2009-17.
26. Wiegand RD, Koutz CA, Chen H, Anderson RE. Effect of dietary fat and environmental lighting on the phospholipid molecular species of rat photoreceptor membranes. Exp Eye Res 1995; 60:291-306.
27. Kang JX, Wang J, Wu L, Kang ZB. Transgenic mice: fat-1 mice convert n-6 to n-3 fatty acids. Nature 2004; 427:504.
28. Spychalla JP, Kinney AJ, Browse J. Identification of an animal omega-3 fatty acid desaturase by heterologous expression in Arabidopsis. Proc Natl Acad Sci USA 1997; 94:1142-7.
29. Tanito M, Brush RS, Elliott MH, Wicker LD, Henry KR, Anderson RE. High levels of retinal membrane docosahexaenoic acid increase susceptibility to stress-induced degeneration. J Lipid Res 2009; 50:807-19.
30. Bligh EG, Dyer WJ. A Rapid Method of Total Lipid Extraction and Purification. Can J Biochem Physiol 1959; 37:911-7.
31. Folch J, Lees M, Stanley GHS. A Simple Method for the Isolation and Purification of Total Lipides from Animal Tissues. J Biol Chem 1957; 226:497-509.
32. Irreverre F, Stone AL, Shichi H, Lewis MS. Biochemistry of visual pigments. I. Purification and properties of bovine rhodopsin. J Biol Chem 1969; 244:529-36.
33. Moriguchi T, Loewke J, Garrison M, Catalan JN, Salem N Jr. Reversal of docosahexaenoic acid deficiency in the rat brain, retina, liver, and serum. J Lipid Res 2001; 42:419-27.
34. Weisinger HS, Vingrys AJ, Sinclair AJ. Effect of dietary n-3 deficiency on the electroretinogram in the guinea pig. Ann Nutr Metab 1996; 40:91-8.
35. Wiegand RD, Koutz CA, Stinson AM, Anderson RE. Conservation of docosahexaenoic acid in rod outer segments of rat retina during n-3 and n-6 fatty acid deficiency. J Neurochem 1991; 57:1690-9.
36. Scott BL, Bazan NG. Membrane docosahexaenoate is supplied to the developing brain and retina by the liver. Proc Natl Acad Sci USA 1989; 86:2903-7.
37. Ambasudhan R, Wang X, Jablonski MM, Thompson DA, Lagali PS, Wong PW, Sieving PA, Ayyagari R. Atrophic macular degeneration mutations in ELOVL4 result in the intracellular misrouting of the protein. Genomics 2004; 83:615-25.
38. Grayson C, Molday RS. Dominant negative mechanism underlies autosomal dominant Stargardt-like macular dystrophy linked to mutations in ELOVL4. J Biol Chem 2005; 280:32521-30.
39. Karan G, Yang Z, Howes K, Zhao Y, Chen Y, Cameron DJ, Lin Y, Pearson E, Zhang K. Loss of ER retention and sequestration of the wild-type ELOVL4 by Stargardt disease dominant negative mutants. Mol Vis 2005; 11:657-64.
40. Weisinger HS, Armitage JA, Jeffrey BG, Mitchell DC, Moriguchi T, Sinclair AJ, Weisinger RS, Salem N Jr. Retinal sensitivity loss in third-generation n-3 PUFA-deficient rats. Lipids 2002; 37:759-65.
41. Agbaga MP, Brush RS, Mandal MN, Henry K, Elliott MH, Anderson RE. Role of Stargardt-3 macular dystrophy protein (ELOVL4) in the biosynthesis of very long chain fatty acids. Proc Natl Acad Sci USA 2008; 105:12843-8.
42. Li C, Cheng M, Yang H, Peachey NS, Naash MI. Age-related changes in the mouse outer retina. Optom Vis Sci 2001; 78:425-30.
43. Lee ES, Flannery JG. Transport of truncated rhodopsin and its effects on rod function and degeneration. Invest Ophthalmol Vis Sci 2007; 48:2868-76.