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Akademický článek

A novel ELOVL4 variant, L168S, causes early childhood-onset Spinocerebellar ataxia-34 and retinal dysfunction: a case report

Autor: Yeboah Kofi Gyening, Keren Boris, Mignot Cyril, Richard S. Brush, Marie-Cécile Nassogne, Martin-Paul Agbaga

Publikováno v: Acta Neuropathologica Communications, Vol 11, Iss 1, Pp 1-10 (2023)

Abstract Spinocerebellar ataxia 34 (SCA34) is an autosomal dominant inherited disease characterized by age-related cerebellar degeneration and ataxia caused by mutations in the Elongation of Very Long Chain Fatty Acid-4 (ELOVL4) gene. The ELOVL4 enzy

Externí odkaz: https://doaj.org/article/56bf065f1de443278b312bf8792439b0

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Akademický článek

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Akademický článek

Salmonids as Natural Functional Food Rich in Omega-3 PUFA

Autor: Wojciech Kolanowski

Publikováno v: Applied Sciences, Vol 11, Iss 5, p 2409 (2021)

Salmonids are valuable fish in the human diet due to their high content of bioactive omega-3 very long-chain polyunsaturated fatty acid (VLC PUFA). The aim of this study was to assess the omega-3 VLC PUFA content in selected salmonid fish present on

Externí odkaz: https://doaj.org/article/f1d6bdf0bcfa460fa882ae58c97720f4

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Biosynthesis of LC-PUFAs and VLC-PUFAs in Pampus argenteus: Characterization of Elovl4 Elongases and Regulation under Acute Salinity

Autor: Juan Carlos Navarro, Kai Liao, Min Jin, Alberto Ribes-Navarro, Jiaxiang Luo, Liangli Xue, Juan Li, Óscar Monroig, Qicun Zhou, Tingting Zhu

Publikováno v: Digital.CSIC. Repositorio Institucional del CSIC
instname
Digital.CSIC: Repositorio Institucional del CSIC
Consejo Superior de Investigaciones Científicas (CSIC)

Salinity has been demonstrated to influence the biosynthesis of long-chain (C20–24) polyunsaturated fatty acids (LC-PUFAs) in teleost fish. Since LC-PUFAs are essential nutrients for vertebrates, it is central to understand how fish cope with an ac

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9746164c1087a06ca6a4eeff284145c2
https://doi.org/10.1021/acs.jafc.0c06277

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Cell Type-Selective Loss of Peroxisomal β-Oxidation Impairs Bipolar Cell but Not Photoreceptor Survival in the Retina

Autor: Daniëlle Swinkels, Yannick Das, Sai Kocherlakota, Stefan Vinckier, Eric Wever, Antoine H.C. van Kampen, Frédéric M. Vaz, Myriam Baes

Publikováno v: Cells
Cells; Volume 11; Issue 1; Pages: 161
Cells, Vol 11, Iss 161, p 161 (2022)
Cells, 11(1):161. MDPI Multidisciplinary Digital Publishing Institute

Retinal degeneration is a common feature in peroxisomal disorders leading to blindness. Peroxisomes are present in the different cell types of the retina; however, their precise contribution to retinal integrity is still unclear. We previously showed

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b91fbe2d1900460265e56211f50e13fe
https://pubmed.ncbi.nlm.nih.gov/35011723

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Very long chain fatty acid-containing lipids: a decade of novel insights from the study of ELOVL4

Autor: Richard S. Brush, Gyening Kofi Yeboah, Martin-Paul Agbaga, Ekaterina S. Lobanova

Publikováno v: Journal of Lipid Research, Vol 62, Iss, Pp 100030-(2021)
Journal of Lipid Research

Lipids play essential roles in maintaining cell structure and function by modulating membrane fluidity and cell signaling. The fatty acid elongase-4 (ELOVL4) protein, expressed in retina, brain, Meibomian glands, skin, testes and sperm, is an essenti

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4bdcd5fd0eef714532da3e8fe1015e72
http://www.sciencedirect.com/science/article/pii/S0022227521000109

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ELOVL4: Very long-chain fatty acids serve an eclectic role in mammalian health and function

Autor: Martin-Paul Agbaga, Robert E. Anderson, Blake R. Hopiavuori

Publikováno v: Progress in retinal and eye research

ELOngation of Very Long chain fatty acids-4 (ELOVL4) is an elongase responsible for the biosynthesis of very long chain (VLC, ≥C28) saturated (VLC-SFA) and polyunsaturated (VLC-PUFA) fatty acids in brain, retina, skin, Meibomian glands, and testes.

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::235f4c6ff3a3e93e11af42a8e60f454a
http://europepmc.org/articles/PMC6688602

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