ArticleProceedings of the National Academy of Sciences of the United States of America2013
Role of ELOVL4 and very long-chain polyunsaturated fatty acids in mouse models of Stargardt type 3 retinal degeneration.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 56 citations in OpenAlex.
- Associations of human retinal very long-chain polyunsaturated fatty acids with dietary lipid biomarkers.Journal of lipid research · 2016Trial
- Age-Driven Lipid Remodeling Activates Lysosome-Mediated Plasma Membrane Repair.Research square · 2026Article
- Testis-specific knockout of Elovl4 reduces sperm motility and fertility in male mice.Scientific reports · 2026Article
- A comprehensive review of the family of very-long-chain fatty acid elongases: structure, function, and implications in physiology and pathology.European journal of medical research · 2023Review
- Understanding the Roles of Very-Long-Chain Polyunsaturated Fatty Acids (VLC-PUFAs) in Eye Health.Nutrients · 2023Review
- Polyunsaturated Lipids in the Light-Exposed and Prooxidant Retinal Environment.Antioxidants (Basel, Switzerland) · 2023Review
- Role of VLC-PUFAs in Retinal and Macular Degeneration.Advances in experimental medicine and biology · 2023Article
- Determination of very long-chain polyunsaturated fatty acids from 24 to 44 carbons in eye, brain and gonads of wild and cultured gilthead sea bream (Sparus aurata).Scientific reports · 2022Article
- Nourishing Better Vision: The ARVO 2021 Mildred Weisenfeld Award Lecture.Investigative ophthalmology & visual science · 2022Article
- Palmitic Acid Lipotoxicity in Microglia Cells Is Ameliorated by Unsaturated Fatty Acids.International journal of molecular sciences · 2021Article
- Lutein and zeaxanthin reduce A2E and iso-A2E levels and improve visual performance in Abca4Experimental eye research · 2021Article
- CIB2 regulates mTORC1 signaling and is essential for autophagy and visual function.Nature communications · 2021Article
- Photoreceptor metabolic reprogramming: current understanding and therapeutic implications.Communications biology · 2021Review
- Retinal bioavailability and functional effects of a synthetic very-long-chain polyunsaturated fatty acid in mice.Proceedings of the National Academy of Sciences of the United States of America · 2021Article
- Identification of Key Genes and Pathways Associated with Age-Related Macular Degeneration.Journal of ophthalmology · 2020Article
- n-3 PUFA Supplementation Alters Retinal Very-Long-Chain-PUFA Levels and Ratios in Diabetic Animal Models.Molecular nutrition & food research · 2019Article
- ELOVL4: Very long-chain fatty acids serve an eclectic role in mammalian health and function.Progress in retinal and eye research · 2019Review
- Article
- Photoreceptor-induced RPE phagolysosomal maturation defects in Stargardt-like Maculopathy (STGD3).Scientific reports · 2018Article
- Retinal de novo lipogenesis coordinates neurotrophic signaling to maintain vision.JCI insight · 2018Article
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
Abstract
Stargardt type 3 (STGD3) disease is a juvenile macular dystrophy caused by mutations in the ELOVL4 (Elongation of very long chain fatty acids 4) gene. Its protein product, ELOVL4, is an elongase required for the biosynthesis of very long-chain polyunsaturated fatty acids (VLC-PUFAs). It is unclear whether photoreceptor degeneration in STGD3 is caused by loss of VLC-PUFAs or by mutated ELOVL4 protein trafficking/aggregation. We therefore generated conditional knockout (cKO) mice with Elovl4 ablated in rods or cones and compared their phenotypes to transgenic (TG) animals that express the human STGD3-causing ELOVL4(STGD3) allele. Gas chromatography-mass spectrometry was used to assess C30-C34 VLC-PUFA and N-retinylidene-N-retinylethanolamine content; electroretinography was used to measure phototransduction and outer retinal function; electron microscopy was used for retinal ultrastructure; and the optomotor tracking response was used to test scotopic and photopic visual performance. Elovl4 transcription and biosynthesis of C30-C34 VLC-PUFAs in rod cKO and TG retinas were reduced up to 98%, whereas the content of docosahexaenoic acid was diminished in TG, but not rod cKO, retinas. Despite the near-total loss of the retinal VLC-PUFA content, rod and cone cKO animals exhibited no electrophysiological or behavioral deficits, whereas the typical rod-cone dystrophic pattern was observed in TG animals. Our data suggest that photoreceptor-specific VLC-PUFA depletion is not sufficient to induce the STGD3 phenotype, because depletion alone had little effect on photoreceptor survival, phototransduction, synaptic transmission, and visual behavior.
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