Cited by 167Fatty Acid Research and HealthRetinal Development and DisordersLipid metabolism and biosynthesis

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Retinal very long-chain PUFAs: new insights from studies on ELOVL4 protein

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Agbaga, Martin-Paul, Mandal, Md Nawajes A., Anderson, Robert E.·2010·Journal of Lipid Research

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Abstract

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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A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Starqardt macular dystrophy
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
P EROXISOMAL β-O XIDATION AND P EROXISOME P ROLIFERATOR –A CTIVATED R ECEPTOR α: An Adaptive Metabolic System
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
Biochemical and functional effects of prenatal and postnatal omega 3 fatty acid deficiency on retina and brain in rhesus monkeys.
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
The key roles of elongases and desaturases in mammalian fatty acid metabolism: Insights from transgenic mice
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
Insights into the Function of Rim Protein in Photoreceptors and Etiology of Stargardt's Disease from the Phenotype in abcr Knockout Mice
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

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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

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Background
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Background
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Background
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Background
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Result
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“Ultralong-chain polyunsaturated fatty acids (PUFAs) have also been found in the other mammalian tissues, such as brain, retina, skin, and testis [107, 108], and 28:5 and 30:5 are essential for sperm maturation and male fertility in the testis [109].”
Background
Paternal low protein diet perturbs inter-generational metabolic homeostasis in a tissue-specific manner in mice
Morgan, H. L., Furse, S., Dias, I. H. K., Shabir, K., Castellanos, M., Khan, I., May, S. T., Holmes, N., Carlile, M., Sang, F., et al. (2022) · Communications Biology · doi:10.1038/s42003-022-03914-8 · Cited by 42
Why Is Zeaxanthin the Most Concentrated Xanthophyll in the Central Fovea?
Widomska, J., SanGiovanni, J. P., & Subczynski, W. K. (2020) · Nutrients · doi:10.3390/nu12051333 · Cited by 37
“The phospholipids containing very-long-chain polyunsaturated fatty acids present in the disk membranes of rod outer segments likely play a unique and important role in the retina because they are necessary for cell survival and their loss leads to cell death [157,158].”
BackgroundMethod
Nano-scale resolution of native retinal rod disk membranes reveals differences in lipid composition
Sander, C. L., Sears, A. E., Pinto, A. F. M., Choi, E. H., Kahremany, S., Gao, F., Salom, D., Jin, H., Pardon, E., Suh, S., et al. (2021) · Journal of Cell Biology · doi:10.1083/jcb.202101063 · Cited by 36
Cholesterol in the rod outer segment: A complex role in a “simple” system
Albert, A. D., Alexander, D., & Boesze‐Battaglia, K. (2016) · Chemistry and Physics of Lipids · doi:10.1016/j.chemphyslip.2016.04.008 · Cited by 37
“Recent studies on the synthesis of very long chain poly unsaturated fatty acids (VLC-PUFA) suggest that VLC-PUFA play important roles in the retina; these fatty acids are over 30 carbons in length with 4 or more double bonds (Agbaga et al., 2010; Bennett and Anderson, 2016).”
Background
Multiple types of programmed necrosis such as necroptosis, pyroptosis, oxytosis/ferroptosis, and parthanatos contribute simultaneously to retinal damage after ischemia–reperfusion
Dvoriantchikova, G., Lypka, K. R., Adis, E. V., & Ivanov, D. (2022) · Scientific Reports · doi:10.1038/s41598-022-22140-0 · Cited by 36
Ablation of retinal ciliopathy protein RPGR results in altered photoreceptor ciliary composition
Rao, K. N., Li, L., Anand, M., & Khanna, H. (2015) · Scientific Reports · doi:10.1038/srep11137 · Cited by 37
Role of ω3 polyunsaturated fatty acids in diabetic retinopathy: a morphological and metabolically cross talk among blood retina barriers damage, autoimmunity and chronic inflammation
Eynard, A. R., & Repossi, G. (2019) · Lipids in Health and Disease · doi:10.1186/s12944-019-1049-9 · Cited by 34
Mutation for Nonsyndromic Mental Retardation in the trans-2-Enoyl-CoA Reductase TER Gene Involved in Fatty Acid Elongation Impairs the Enzyme Activity and Stability, Leading to Change in Sphingolipid Profile
Abe, K., Ohno, Y., Sassa, T., Taguchi, R., Çalışkan, M., Ober, C., & Kihara, A. (2013) · Journal of Biological Chemistry · doi:10.1074/jbc.m113.493221 · Cited by 34
“2 Although much less abundant than long-chain (C11-C20) FAs (LCFAs), VLCFAs are ubiquitous among tissues and play an important function that cannot be substituted for by LCFAs, including skin barrier formation, neural function, retina function, and resolution of inflammation (1-3).”
Background
ELOVL2: Not just a biomarker of aging
Chao, D. L., & Skowronska‐Krawczyk, D. (2020) · Translational Medicine of Aging · doi:10.1016/j.tma.2020.06.004 · Cited by 33
Advances in Analyzing the Breast Cancer Lipidome and Its Relevance to Disease Progression and Treatment
Ward, A. V., Anderson, S. M., & Sartorius, C. A. (2021) · Journal of Mammary Gland Biology and Neoplasia · doi:10.1007/s10911-021-09505-3 · Cited by 35
“Chain length typically ranges from 4–22 carbons; however, chains with > 24 carbons are occasionally present in mammals [21].”
Background
Prevalence and clinicoradiological features of spinocerebellar ataxia type 34 in a Japanese ataxia cohort
Ozaki, K., Ansai, A., Nobuhara, K., Araki, T., Kubodera, T., Ishii, T., Higashi, M., Sato, N. J., Soga, K., Mizusawa, H., et al. (2019) · Parkinsonism & Related Disorders · doi:10.1016/j.parkreldis.2019.05.019 · Cited by 31
Membrane‐type frizzled‐related protein regulates lipidome and transcription for photoreceptor function
Kautzmann, M.-A. I., Gordon, W. C., Jun, B., Do, K. V., Matherne, B., Fang, Z., & Bazan, N. G. (2019) · The FASEB Journal · doi:10.1096/fj.201902359r · Cited by 30
Inherited disorders of complex lipid metabolism: A clinical review
Xiao, C., Rossignol, F., Vaz, F. M., & Ferreira, C. R. (2021) · Journal of Inherited Metabolic Disease · doi:10.1002/jimd.12369 · Cited by 31
Effects on vesicular transport pathways at the late endosome in cells with limited very long-chain fatty acids
Obara, K., Kojima, R., & Kihara, A. (2013) · Journal of Lipid Research · doi:10.1194/jlr.m034678 · Cited by 31
“Mammalian polyunsaturated VLCFAs are used in the generation of glycerolipids and precursors of lipid mediators, which have many physiological roles in cellular processes such as resolution of inflammation and retinal functions (3-5).”
Serum levels of lipid metabolites in age‐related macular degeneration
Orban, T., Johnson, W. M., Dong, Z., Maeda, T., Maeda, A., Sakai, T., Tsuneoka, H., Mieyal, J. J., & Palczewski, K. (2015) · The FASEB Journal · doi:10.1096/fj.15-275289 · Cited by 29
“However, very-long-chain polyunsaturated fatty acids (whose production is catalyzed by ELOVL-4) are similar to DHA because of their role in photoreceptor function and integrity (63).”
Background
Isolation and Characterization of the Diatom Phaeodactylum Δ5-Elongase Gene for Transgenic LC-PUFA Production in Pichia pastoris
Jiang, M., Guo, B., Wan, X., Gong, Y., Zhang, Y., & Hu, C. (2014) · Marine Drugs · doi:10.3390/md12031317 · Cited by 29
“The n-3 PUFAs including DHA and EPA can be used as preventive drugs and supplements [3–6].”
Cloning and functional characterization of fads2 desaturase and elovl5 elongase from Japanese flounder Paralichthys olivaceus
Kabeya, N., Chiba, M., Haga, Y., Satoh, S., & Yoshizaki, G. (2017) · Comparative Biochemistry and Physiology Part B Biochemistry and Molecular Biology · doi:10.1016/j.cbpb.2017.09.002 · Cited by 29
“Since these tissues a major metabolic site for LCPUFA (Agbaga et al., 2010), it is reasonable that both genes show relatively high expressions.”
Background
Human Breast Milk: The Key Role in the Maturation of Immune, Gastrointestinal and Central Nervous Systems: A Narrative Review
Dimitroglou, M., Iliodromiti, Z., Christou, E., Volaki, P., Petropoulou, C., Sokou, R., Boutsikou, Τ., & Iacovidou, N. M. (2022) · Diagnostics · doi:10.3390/diagnostics12092208 · Cited by 28
Endoplasmic reticulum microenvironment and conserved histidines govern ELOVL4 fatty acid elongase activity
Logan, S., Agbaga, M., Chan, M. D., Brush, R. S., & Anderson, R. E. (2014) · Journal of Lipid Research · doi:10.1194/jlr.m045443 · Cited by 28
Overview of how N32 and N34 elovanoids sustain sight by protecting retinal pigment epithelial cells and photoreceptors
Bazan, N. G. (2020) · Journal of Lipid Research · doi:10.1194/jlr.tr120001137 · Cited by 27
“ELOVL4 catalyzes the synthesis of VLC-PUFAs in the retina (10, 21) and testes (22) and generates VLC-saturated FAs in the skin and brain (23, 24).”
Background
Two Modes of Regulation of the Fatty Acid Elongase ELOVL6 by the 3-Ketoacyl-CoA Reductase KAR in the Fatty Acid Elongation Cycle
Naganuma, T., & Kihara, A. (2014) · PLoS ONE · doi:10.1371/journal.pone.0101823 · Cited by 29
“Saturated and monounsaturated VLCFAs are specifically used for sphingolipid production [2,3,4], whereas most polyunsaturated VLCFAs are incorporated into glycerophospholipids [5,6,7].”
Background
Hetero-oligomeric interactions of an ELOVL4 mutant protein: implications in the molecular mechanism of Stargardt-3 macular dystrophy.
Okuda, A., Naganuma, T., Ohno, Y., Abe, K., Yamagata, M., Igarashi, Y., & Kihara, A. (2010) · PubMed Central · Cited by 26
Factors Differentiating the Antioxidant Activity of Macular Xanthophylls in the Human Eye Retina
Widomska, J., Gruszecki, W. I., & Subczynski, W. K. (2021) · Antioxidants · doi:10.3390/antiox10040601 · Cited by 25
Oral Metformin Inhibits Choroidal Neovascularization by Modulating the Gut-Retina Axis
Zhang, J. Y., Xiao, J. F., Xie, B., Barba, H., Boachie-Mensah, M., Shah, R. N., Nadeem, U., Spedale, M., Dylla, N. P., Lin, H., et al. (2023) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.64.15.21 · Cited by 25
Retinal de novo lipogenesis coordinates neurotrophic signaling to maintain vision
Rajagopal, R., Zhang, S., Wei, X., Doggett, T. A., Adak, S., Enright, J. M., Shah, V., Ling, G., Chen, S., Yoshino, J., et al. (2018) · JCI Insight · doi:10.1172/jci.insight.97076 · Cited by 23
“In particular, docosahexaenoic acid–containing (DHA-containing) phospholipids are abundant (28), and these species are preferentially localized to specialized membrane structures, such as synaptic membranes.”
Background
The ELOVL proteins: Very and ultra long-chain fatty acids at the crossroads between metabolic and neurodegenerative disorders.
Ferrero, E., Vaz, F. M., Cheillan, D., Brusco, A., & Marelli, C. (2025) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2025.109050 · Cited by 24
“…and belong to the ω 3 and ω 6 families [32], are incorporated into phosphatidylcholines at the sn-1 position, with DHA at the sn-2 position; these ULCFA-containing phosphatidylcholines in the retinal outer segment membrane of photo-receptors are crucial for phototransduction [33][34].”
Understanding the Roles of Very-Long-Chain Polyunsaturated Fatty Acids (VLC-PUFAs) in Eye Health
Nwagbo, U., & Bernstein, P. S. (2023) · Nutrients · doi:10.3390/nu15143096 · Cited by 26
“They have a hybrid structure combining a proximal carboxylic end characteristic of saturated fatty acids and a distal end containing methylene-interrupted cis double bonds [2] (Figure 1).”
BackgroundInfluential
Identification of very long-chain (>C24) fatty acid methyl esters using gas chromatography coupled to quadrupole/time-of-flight mass spectrometry with atmospheric pressure chemical ionization source
Garlito, B., Portolés, T., Niessen, W. M. A., Navarro, J. C., Hontoria, F., Monroig, Ó., Varó, I., & Serrano, R. (2018) · Analytica Chimica Acta · doi:10.1016/j.aca.2018.11.001 · Cited by 25
Peroxisomal Disorders and Their Mouse Models Point to Essential Roles of Peroxisomes for Retinal Integrity
Das, Y., Swinkels, D., & Baes, M. I. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22084101 · Cited by 24
The catadromous teleost Anguilla japonica has a complete enzymatic repertoire for the biosynthesis of docosahexaenoic acid from α-linolenic acid: Cloning and functional characterization of an Elovl2 elongase
Xu, W., Wang, S., You, C., Zhang, Y., Monroig, Ó., Tocher, D. R., & Li, Y. (2019) · Comparative Biochemistry and Physiology Part B Biochemistry and Molecular Biology · doi:10.1016/j.cbpb.2019.110373 · Cited by 23
Biosynthesis of Long-Chain Polyunsaturated Fatty Acids in Marine Gammarids: Molecular Cloning and Functional Characterisation of Three Fatty Acyl Elongases
Ribes‐Navarro, A., Navarro, J. C., Hontoria, F., Kabeya, N., Standal, I. B., Evjemo, J. O., & Monroig, Ó. (2021) · Marine Drugs · doi:10.3390/md19040226 · Cited by 24
“Analysis of VLC-PUFAs from natural samples is technically challenging [56,57] and it can be only accurately performed on lipid samples prepared from specific polar lipid fractions collected from key tissues such as the CNS or retina.”
Background
Bioavailability and spatial distribution of fatty acids in the rat retina after dietary omega-3 supplementation
Vidal, E., Jun, B., Gordon, W. C., Maire, M., Martine, L., Gregoire, S., Khoury, S., Cabaret, S., Berdeaux, O., Acar, N., et al. (2020) · Journal of Lipid Research · doi:10.1194/jlr.ra120001057 · Cited by 23
“(37–40) or retinal diseases (41, 42) because they share similar mechanical features with humans.”
Background
Molecular and Functional Characterization of Elovl4 Genes in Sparus aurata and Solea senegalensis Pointing to a Critical Role in Very Long-Chain (>C24) Fatty Acid Synthesis during Early Neural Development of Fish
Morais, S., Torres-Rodríguez, M., Hontoria, F., Monroig, Ó., Varó, I., Agulleiro, M. J., & Navarro, J. C. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21103514 · Cited by 22
“From what is known in higher vertebrates, VLC-PUFA are generally incorporated into phosphatidylcholine in the photoreceptor cells that make up the retina [7], and are then bioconverted into elovanoids, which participate in photoreceptor protection [8,13].”
BackgroundResultInfluential
An SPM-Enriched Marine Oil Supplement Shifted Microglia Polarization toward M2, Ameliorating Retinal Degeneration in rd10 Mice
Olivares‐González, L., Velasco, S., Gallego, I., Esteban‐Medina, M., Puras, G., Loucera, C., Martínez‐Romero, A., Peña-Chilet, M., Pedraz, J. L., & Rodrigo, R. (2022) · Antioxidants · doi:10.3390/antiox12010098 · Cited by 21
“Furthermore, retinal elongases, especially ELOVL4, synthetize very-long-chain PUFAs (VLC-PUFAs) [14] from DHA, which are converted in elovanoids (PD analogues) in response to oxidative stress protecting PRs and RPE cells [15].”
Background
The Transcriptional Factor PPARαb Positively Regulates Elovl5 Elongase in Golden Pompano Trachinotus ovatus (Linnaeus 1758)
Zhu, K.-C., Ling, S., Zhao, C.-P., Guo, H., Zhang, N., Guo, L. J., Liu, B.-S., Jiang, S., & Zhang, D. (2018) · Frontiers in Physiology · doi:10.3389/fphys.2018.01340 · Cited by 21
Analysis of Lipid Peroxidation by UPLC-MS/MS and Retinoprotective Effects of the Natural Polyphenol Pterostilbene
Torres-Cuevas, I., Millán, I., Asensi, M. A., Vento, M., Oger, C., Galano, J., Durand, T., & Ortega, Á. (2021) · Antioxidants · doi:10.3390/antiox10020168 · Cited by 21
“The retina is one of the body tissues with the highest concentration of long-chain polyunsaturated fatty acids (PUFAs), the latter being essential for the maintenance of the physiological retinal function and development [10,11].”
Background
Peroxisomal Multifunctional Protein 2 Deficiency Perturbs Lipid Homeostasis in the Retina and Causes Visual Dysfunction in Mice
Das, Y., Swinkels, D., Kocherlakota, S., Vinckier, S., Vaz, F. M., Wever, E. J. M., van Kampen, A. H. C., Jun, B., Do, K. V., Moons, L., et al. (2021) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2021.632930 · Cited by 21
“The marked accumulation of PUFAs with ultra-long chains (≥ C34) raised the question whether this was related to increased expression of the Elovl4 gene in the retina, given its role in the elongation of fatty acids containing ≥ 26 carbons (Agbaga et al., 2010).”
BackgroundInfluential
Cardiac dysfunction affects eye development and vision by reducing supply of lipids in fish
Sørhus, E., Meier, S., Donald, C. E., Furmanek, T., Edvardsen, R. B., & Lie, K. K. (2021) · The Science of The Total Environment · doi:10.1016/j.scitotenv.2021.149460 · Cited by 20
Dysfunctional peroxisomal lipid metabolisms and their ocular manifestations
Chen, C. T., Shao, Z., & Fu, Z. (2022) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2022.982564 · Cited by 19
Inducible RPE-specific GPX4 knockout causes oxidative stress and retinal degeneration with features of age-related macular degeneration
Wojciechowski, A. M., Bell, B. A., Song, Y., Anderson, B. D., Conomikes, A., Petruconis, C. R., & Dunaief, J. L. (2024) · Experimental Eye Research · doi:10.1016/j.exer.2024.110028 · Cited by 20
Molecular Species of Phospholipids with Very Long Chain Fatty Acids in Skin Fibroblasts of Zellweger Syndrome
Hama, K., Nagai, T., 西澤, 千., Ikeda, K., Morita, M., Satoh, N., Nakanishi, H., Imanaka, T., Shimozawa, N., Taguchi, R., et al. (2013) · Lipids · doi:10.1007/s11745-013-3848-5 · Cited by 21
“Polyunsaturated VLCFA are usually found in the retina and testis of normal mammals, including humans [3, 4].”
Background
Etiology and treatment of adrenoleukodystrophy: new insights from Drosophila
Gordon, H. B., Valdez, L., & Letsou, A. (2018) · Disease Models & Mechanisms · doi:10.1242/dmm.031286 · Cited by 22
“Interestingly, in humans, the longest VLCFA species are found only in tissues expressing elongases, namely the retina, brain and testis, all three of which are key tissues affected in ALD (Agbaga et al., 2010; Ahmad et al., 2009).”
Evaluating Ultra-long-Chain Fatty Acids as Biomarkers of Colorectal Cancer Risk
Perttula, K., Edmands, W. M. B., Grigoryan, H., Cai, X., Iavarone, A. T., Gunter, M. J., Naccarati, A., Polidoro, S., Hubbard, A., Vineis, P., et al. (2016) · Cancer Epidemiology Biomarkers & Prevention · doi:10.1158/1055-9965.epi-16-0204 · Cited by 18
Bioengineered Plants Can Be an Alternative Source of Omega-3 Fatty Acids for Human Health
Lakra, N., Mahmood, S., Marwal, A., M., S. N., & Anwar, K. (2019) · Springer eBooks · doi:10.1007/978-3-030-03344-6_16 · Cited by 18
Expression of genes related to long-chain (C18–22) and very long-chain (>C24) fatty acid biosynthesis in gilthead seabream (Sparus aurata) and Senegalese sole (Solea senegalensis) larvae: investigating early ontogeny and nutritional regulation
Torres-Rodríguez, M., Navarro, J. C., Varó, I., Agulleiro, M. J., Morais, S., Monroig, Ó., & Hontoria, F. (2020) · Aquaculture · doi:10.1016/j.aquaculture.2020.734949 · Cited by 19
Nutritional regulation of genes responsible for long-chain (C20-24) and very long-chain (>C24) polyunsaturated fatty acid biosynthesis in post-larvae of gilthead seabream (Sparus aurata) and Senegalese sole (Solea senegalensis)
Torres-Rodríguez, M., Navarro, J. C., Varó, I., Monroig, Ó., & Hontoria, F. (2020) · Aquaculture · doi:10.1016/j.aquaculture.2020.735314 · Cited by 18
The Elovl4 Spinocerebellar Ataxia-34 Mutation 736T>G (p.W246G) Impairs Retinal Function in the Absence of Photoreceptor Degeneration
Agbaga, M., Stiles, M. A., Brush, R. S., Sullivan, M. T., Machalinski, A. H., Jones, K. L., Anderson, R. E., & Sherry, D. M. (2020) · Molecular Neurobiology · doi:10.1007/s12035-020-02052-8 · Cited by 18
“ELOVL4 is the only known fatty acid elongase that performs this function [1, 2].”
Background

Authors: Agbaga, M.-P., Mandal, M. N. A., & Anderson, R. E.
Affiliations: Dean McGee Eye Institute (US)
Corresponding author: Anderson, Robert E.
ORCID: Agbaga, Martin-Paul
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)
DOI: 10.1194/jlr.r005025
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: cc-by
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
creativecommons.org/licenses/by/4.0/ (vor, 3744 d)
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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Retinal very long-chain PUFAs: new insights from studies on ELOVL4 protein

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