The DOI matches its Crossref record; 240 of the 254 references it cites were found in a registry or an index.
Retinal very long-chain PUFAs: new insights from studies on ELOVL4 protein
Open accessAbstract
Compared with other mammalian tissues, retina is highly enriched in PUFA. Long-chain PUFA (LC-PUFA; C18-C24) are essential FAs that are enriched in the retina and are necessary for maintenance of normal retinal development and function. The retina, brain, and sperm also contain very LC-PUFA (VLC-PUFA; >C24). Although VLC-PUFA were discovered more than two decades ago, very little is known about their biosynthesis and functional roles in the retina. This is due mainly to intrinsic difficulties associated with working on these unusually long polyunsaturated hydrocarbon chains and their existence in small amounts. Recent studies on the FA elongase elongation of very long chain fatty acids-4 (ELOVL4) protein, however, suggest that VLC-PUFA probably play some uniquely important roles in the retina as well as the other tissues. Mutations in the ELOVL4 gene are found in patients with autosomal dominant Stargardt disease. Here, we review the recent literature on VLC-PUFA with special emphasis on the elongases responsible for their synthesis. We focus on a novel elongase, ELOVL4, involved in the synthesis of VLC-PUFA, and the importance of these FAs in maintaining the structural and functional integrity of retinal photoreceptors.
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Allikmets, R., Singh, N., Sun, H., Shroyer, N. F., Hutchinson, A., Chidambaram, A., Gerrard, B., Baird, L., Stauffer, D., Peiffer, A., et al. (1997) · Nature Genetics · doi:10.1038/ng0397-236 · Cited by 1,435
Dietary polyunsaturated fatty acids and inflammatory mediator production
James, M. J., Gibson, R. A., & Cleland, L. G. (2000) · American Journal of Clinical Nutrition · doi:10.1093/ajcn/71.1.343s · Cited by 1,129
Chemistry and metabolism of lipids in the vertebrate retina
Fliesler, A. J., & Anderson, R. E. (1983) · Progress in Lipid Research · doi:10.1016/0163-7827(83)90004-8 · Cited by 1,052
Reddy, J. K., & Hashimoto, T. (2001) · Annual Review of Nutrition · doi:10.1146/annurev.nutr.21.1.193 · Cited by 991
Fatty acid elongases in mammals: their regulation and roles in metabolism
JAKOBSSON, A., WESTERBERG, R., & JACOBSSON, A. (2006) · Progress in Lipid Research · doi:10.1016/j.plipres.2006.01.004 · Cited by 936
Neuringer, M. D., CONNOR, W. E., Lin, D. S., Barstad, L., & Luck, S. (1986) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.83.11.4021 · Cited by 914
Guillou, H., Zadravec, D., Martin, P. G. P., & Jacobsson, A. (2009) · Progress in Lipid Research · doi:10.1016/j.plipres.2009.12.002 · Cited by 913
The role of omega-3 long-chain polyunsaturated fatty acids in health and disease of the retina
SanGiovanni, J. P., & Chew, E. Y. (2004) · Progress in Retinal and Eye Research · doi:10.1016/j.preteyeres.2004.06.002 · Cited by 905
Weng, J., Mata, N. L., Azarian, S. M., Tzekov, R. T., Birch, D. G., & Travis, G. H. (1999) · Cell · doi:10.1016/s0092-8674(00)80602-9 · Cited by 881
Mutation of the Stargardt Disease Gene ( ABCR ) in Age-Related Macular Degeneration
Allikmets, R. L., Shroyer, N. F., Singh, N., Seddon, J. M., Lewis, R. A., Bernstein, P. S., Peiffer, A. P., Zabriskie, N. A., Li, Y., Hutchinson, A. A., et al. (1997) · Science · doi:10.1126/science.277.5333.1805 · Cited by 879
Research works printed but not linked: 3
- Butovich, 2009, Human tear film and meibum. Very long chain wax esters and (O-acyl)-omega-hydroxy fatty acids of meibum, J. Lipid Res., 50, 2471, 10.1194/jlr.M900252-JLR200 · DOI
- Mitchell, 2007, Effects of ROS disk membrane phospholipids with extremely long polyunsaturated acyl chains on visual signalling, Invest. Ophthalmol. Vis. Sci., 48, 2928
- Liu, 2009, Enhanced methods for analysis of very long chain polyunsaturated fatty acids from retina and RPE, Invest. Ophthalmol. Vis. Sci., 50, 3410
Cited by
Sorted by citations
Dyall, S. C., Balas, L., Bazan, N. G., Brenna, J. T., Chiang, N., Souza, F. D. C., Dalli, J., Durand, T., Galano, J., Lein, P. J., et al. (2022) · Progress in Lipid Research · doi:10.1016/j.plipres.2022.101165 · Cited by 707
“In photoreceptor cells, VLC-PUFAs are in phosphatidylcholines (PC) of the outer segment membranes, tightly bound to rhodopsin [183].”
BackgroundMetabolism and functional effects of plant-derived omega-3 fatty acids in humans
Baker, E. J., Miles, E. A., Burdge, G. C., Yaqoob, P., & Calder, P. C. (2016) · Progress in Lipid Research · doi:10.1016/j.plipres.2016.07.002 · Cited by 500
Castro, L. F. C., Tocher, D. R., & Monroig, Ó. (2016) · Progress in Lipid Research · doi:10.1016/j.plipres.2016.01.001 · Cited by 461
Very long-chain fatty acids: elongation, physiology and related disorders
Kihara, A. (2012) · The Journal of Biochemistry · doi:10.1093/jb/mvs105 · Cited by 453
“to six double bonds at the sn 1 position (13, 34).”
BackgroundInfluentialMetabolism of Very Long-Chain Fatty Acids: Genes and Pathophysiology
Sassa, T., & Kihara, A. (2014) · Biomolecules & Therapeutics · doi:10.4062/biomolther.2014.017 · Cited by 302
“ULC-PUFAs are found in the testis, brain, and retina (Agbaga et al., 2010).”
Metadata
- Authors
- Agbaga, M.-P., Mandal, M. N. A., & Anderson, R. E.
- Affiliations
- Dean McGee Eye Institute (US)
- Corresponding author
- Anderson, Robert E.
- Venue
- Journal of Lipid Research
- Publisher
- Elsevier BV
- Published
- 2010-07
- Volume
- 51
- Issue
- 7
- Pages
- 1624-1642
- ISSN
- 0022-2275 (ISSN-L)
- PMC
- PMC2882734
- OpenAlex
- W2137994863
- Cited by
- 167
- References
- 254
- Citations by year
- 2026: 2 · 2025: 11 · 2024: 8 · 2023: 12 · 2022: 11 · 2021: 19 · 2020: 13 · 2019: 12 · 2018: 15 · 2017: 12 · 2016: 10 · 2015: 6 · 2014: 9 · 2013: 7 · 2012: 7
- FWCI
- 0.97
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Lipid Research (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Eye Institute (P30EY012190, EY-12190, EY04149, R01EY000871, R01EY004149, EY 00871)
- National Center for Research Resources (RR 17703, P20RR017703)
- Topics
- Health Sciences › Nursing › Nutrition and Dietetics › Fatty Acid Research and Health
- Health Sciences › Medicine › Ophthalmology › Retinal Development and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
- Keywords
- very long chain polyunsaturated fatty acids, ELOVL4, fatty acid elongation, retinal lipids, Stargardt disease, photoreceptors, retinal development, fatty acid synthesis, essential fatty acids
- MeSH terms
- Fatty Acid Elongases, Acetyltransferases, Animals, Cell Membrane, DNA Mutational Analysis, Eye Proteins, Fatty Acids, Unsaturated, Humans, Membrane Proteins, Retina, Retinaldehyde, Substrate Specificity, Tissue Distribution, Molecular Structure
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