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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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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