ArticleThe Journal of biological chemistry2012
Essential role of ELOVL4 protein in very long chain fatty acid synthesis and retinal function.
Article in The Journal of biological chemistry, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 49 papers.
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Who cites it
49 citing papers in PubMed, 92 citations in OpenAlex.
- Associations of human retinal very long-chain polyunsaturated fatty acids with dietary lipid biomarkers.Journal of lipid research · 2016Trial
- Identification of key biomarkers of telomere-related genes in diabetic nephropathy via bioinformatic analysis.Frontiers in genetics · 2026Article
- Rod-specific downregulation of omega-3 very-long-chain polyunsaturated fatty acid pathway in age-related macular degeneration.Experimental eye research · 2023Article
- Understanding the Roles of Very-Long-Chain Polyunsaturated Fatty Acids (VLC-PUFAs) in Eye Health.Nutrients · 2023Review
- A genome-wide association study for the fatty acid composition of breast meat in an F2 crossbred chicken population.Journal of animal science and technology · 2023Article
- Incomplete Elongation of Ultra-long-chain Polyunsaturated Acyl-CoAs by the Fatty Acid Elongase ELOVL4 in Spinocerebellar Ataxia Type 34.Molecular and cellular biology · 2023Article
- Stargardt-like Clinical Characteristics and Disease Course Associated with Variants in theGenes · 2023Article
- Influence of very-long-chain polyunsaturated fatty acids on membrane structure and dynamics.Biophysical journal · 2022Article
- Coenzyme Q10 in the eye isomerizes by sunlight irradiation.Scientific reports · 2022Article
- Transcriptional analysis of microRNAs related to unsaturated fatty acid synthesis by interfering bovine adipocyte ACSL1 gene.Frontiers in genetics · 2022Article
- Dysfunctional peroxisomal lipid metabolisms and their ocular manifestations.Frontiers in cell and developmental biology · 2022Review
- RNA-Seq Analysis Reveals the Potential Molecular Mechanisms of Puerarin on Intramuscular Fat Deposition in Heat-Stressed Beef Cattle.Frontiers in nutrition · 2022Article
- Upregulation of the PPAR signaling pathway and accumulation of lipids are related to the morphological and structural transformation of the dragon-eye goldfish eye.Science China. Life sciences · 2021Article
- The structural basis of fatty acid elongation by the ELOVL elongases.Nature structural & molecular biology · 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
- Lipid Landscape of the Human Retina and Supporting Tissues Revealed by High-Resolution Imaging Mass Spectrometry.Journal of the American Society for Mass Spectrometry · 2020Article
- Bioavailability and spatial distribution of fatty acids in the rat retina after dietary omega-3 supplementation.Journal of lipid research · 2020Article
- Recent advances in the mass spectrometric analysis of glycosphingolipidome - A review.Analytica chimica acta · 2020Review
- Aging membranes: Unexplored functions for lipids in the lifespan of the central nervous system.Experimental gerontology · 2020Review
Corrections and comments
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Authors and funding
17 authors at 6 institutions in 3 countries.
Funding
Abstract
Very long chain polyunsaturated fatty acid (VLC-PUFA)-containing glycerophospholipids are highly enriched in the retina; however, details regarding the specific synthesis and function of these highly unusual retinal glycerophospholipids are lacking. Elongation of very long chain fatty acids-4 (ELOVL4) has been identified as a fatty acid elongase protein involved in the synthesis of VLC-PUFAs. Mutations in ELOVL4 have also been implicated in an autosomal dominant form of Stargardt disease (STGD3), a type of juvenile macular degeneration. We have generated photoreceptor-specific conditional knock-out mice and used high performance liquid chromatography-mass spectrometry (HPLC-MS) to examine and analyze the fatty acid composition of retinal membrane glycerophosphatidylcholine and glycerophosphatidylethanolamine species. We also used immunofluorescent staining and histology coupled with electrophysiological data to assess retinal morphology and visual response. The conditional knock-out mice showed a significant decrease in retinal glycerophospholipids containing VLC-PUFAs, specifically contained in the sn-1 position of glycerophosphatidylcholine, implicating the role of Elovl4 in their synthesis. Conditional knock-out mice were also found to have abnormal accumulation of lipid droplets and lipofuscin-like granules while demonstrating photoreceptor-specific abnormalities in visual response, indicating the critical role of Elovl4 for proper rod or cone photoreceptor function. Altogether, this study demonstrates the essential role of ELOVL4 in VLC-PUFA synthesis and retinal function.
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