Impaired synthesis of DHA in patients with X-linked retinitis pigmentosa

Journal of Lipid Research

Volumn 42, Issue 9, 2001, Pages 1395-1401

  Hoffman, Dennis R ^a   Demar, James C ^b   Heird, William C ^b   Birch, David G ^a   Anderson, Robert E ^c  

^a RETINA FOUNDATION OF THE SOUTHWEST   (United States)

^b BAYLOR COLLEGE OF MEDICINE   (United States)

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

Author keywords

Delta 5 desaturase; Linolenic acid; Lipid metabolism; Omega 3 fatty acids; Retinal degeneration; Stable isotopes

Indexed keywords

DOCOSAHEXAENOIC ACID; OMEGA 3 FATTY ACID;

ADULT; ARTICLE; BIOSYNTHESIS; CLINICAL ARTICLE; CONTROLLED STUDY; FATTY ACID SYNTHESIS; HUMAN; LIPID METABOLISM; MALE; MASS SPECTROMETRY; PATHOGENESIS; PRIORITY JOURNAL; RETINA DEGENERATION; RETINITIS PIGMENTOSA;

ADULT; ALPHA-LINOLENIC ACID; BREATH TESTS; CARBON DIOXIDE; CARBON ISOTOPES; DOCOSAHEXAENOIC ACIDS; FATTY ACID DESATURASES; FATTY ACIDS, OMEGA-3; GAS CHROMATOGRAPHY-MASS SPECTROMETRY; HUMANS; KINETICS; LINKAGE (GENETICS); MALE; MIDDLE AGED; OXIDATION-REDUCTION; RETINITIS PIGMENTOSA; X CHROMOSOME;

EID: 0035652063     PISSN: 00222275     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (43)

1. X-linked retinitis pigmentosa
2. X-linked retinitis pigmentosa. Profile of clinical findings
3. Early abnormalities of rod function in children with X-linked retinitis pigmentosa
4. Fatty acids and visual dysfunction
5. Red blood cell membrane phosphatidylethanolamine fatty acid content in various forms of retinitis pigmentosa
6. Docosahexaenoic acid in red blood cells of patients with X-linked retinitis pigmentosa
7. Pathogenesis of progressive rod-cone degeneration in miniature poodles
8. Plasma lipid abnormalities in the miniature poodle with progressive rod-cone degeneration
9. A hypothesis to explain the reduced blood levels of docosahexaenoic acid in inherited retinal degenerations caused by mutations in genes encoding retina-specific proteins
10. Diets enriched in docosahexaenoic acid fail to correct progressive rodcone degeneration (prcd) phenotype
11. Lower ROS docosahexaenoic acid phenotype in rats and mice with rhodopsin mutations
12. Chemistry and metabolism of lipids in the vertebrate retina
13. Effect of docosahexaenoic acid on membrane fluidity and function in intact cultured Y-79 retinoblastoma cells
14. A role for phospholipid polyunsaturation in modulating membrane protein function
15. Membrane fatty acids associated with the electrical response in visual excitation
16. Visual membranes: Specificity of fatty acid precursors for the electrical response to illumination
17. Effect of dietary alpha-linolenate/linoleate balance on the lipid composition and electroretinographic responses in rats
18. The effects of dietary α-linolenic acid on the composition of nerve membranes, enzymatic activity, amplitude of electrophysiological parameters, resistance to poisons and performance of learning tasks in rats
19. Effect of dietary n-3 deficiency on the electroretinogram in the guinea pig
20. Dietary, omega-3 fatty acid deficiency and visual loss in infant rhesus monkeys
21. n-3 Fatty acids in the brain and retina: Evidence for their essentiality
22. Biochemical and functional effects of prenatal and postnatal ω3 fatty acid deficiency on retina and brain in rhesus monkeys
23. Effect of dietary omega-3 fatty acids on retinal function of very-low-birth-weight neonates
24. Retinal development in very-low-birth-weight infants fed diets differing in omega-3 fatty acids
25. Dietary essential fatty acid supply and visual acuity development
26. Effect of long-chain n-3 fatty acid supplementation on visual acuity and growth of preterm infants with and without bronchopulmonary dysplasia
27. Dietary essential fatty acids, long-chain polyunsaturated fatty acids, and visual resolution acuity in healthy fullterm infants: A systematic review
28. A randomized controlled trial of early dietary supply of long chain polyunsaturated fatty acids and mental development in term infants
29. Dietary requirements and function of α-linolenic acid in animals
30. The metabolism of 7, 10, 13, 16, 19-docosapentaenoic acid to 4, 7, 10, 13, 16, 19-docosahexaenoic acid in rat liver is independent of a 4-desaturase
31. Regulation of the biosynthesis of 4,7,10,13,16,19-docosahexaenoic acid
32. Synthesis of docosahexaenoic acid by retina and retinal pigment epithelium
33. A comparison of the metabolism of [3-14C]-labeled 22- and 24-carbon (n-3) and (n-6) unsaturated fatty acids by rat testes and liver
34. A rapid method of total lipid extraction and purification
35. Preparation of fatty acid methyl esters and dimethyl acetals from lipids with boron tri-fluoride-methanol
36. Isotopic determination of organic keto acid pentafluorobenzyl esters in biological fluids by negative chemical ionization gas chromatography/mass spectrometry
37. Polyunsaturated fatty acid biosynthesis: A microsomal-peroxisomal process
38. Metabolism of omega-3 fatty acids in patients with autosomal dominant retinitis pigmentosa
39. Alterations in plasma lipoproteins and DHA transport in progressive rod-cone degeneration (prcd)
40. Altered desaturase activities and fatty acid composition in liver microsomes of spontaneously diabetic Wistar BB rat
41. Glucocorticoid and mineralcorticoid hormones depress liver delta 5-desaturase activity through different mechanisms
42. Dietary lipids influence the activity of delta 5-desaturase and phospholipid fatty acids in rat enterocyte microsomal membranes