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http://hdl.handle.net/2445/178699
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DC Field | Value | Language |
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dc.contributor.author | Mirra, Serena | - |
dc.contributor.author | García-Arroyo, Rocío | - |
dc.contributor.author | Domènech, Elena B. | - |
dc.contributor.author | Gavaldà i Navarro, Aleix | - |
dc.contributor.author | Herrera Úbeda, Carlos | - |
dc.contributor.author | Oliva, Clara | - |
dc.contributor.author | Garcia Fernández, Jordi | - |
dc.contributor.author | Artuch Iriberri, Rafael | - |
dc.contributor.author | Villarroya i Gombau, Francesc | - |
dc.contributor.author | Marfany i Nadal, Gemma | - |
dc.date.accessioned | 2021-06-28T12:56:05Z | - |
dc.date.available | 2022-05-25T05:10:21Z | - |
dc.date.issued | 2021-05-25 | - |
dc.identifier.issn | 0969-9961 | - |
dc.identifier.uri | http://hdl.handle.net/2445/178699 | - |
dc.description.abstract | The retina is a highly active metabolic organ that displays a particular vulnerability to genetic and environmental factors causing stress and homeostatic imbalance. Mitochondria constitute a bioenergetic hub that coordinates stress response and cellular homeostasis, therefore structural and functional regulation of the mitochondrial dynamic network is essential for the mammalian retina. CERKL (ceramide kinase like) is a retinal degeneration gene whose mutations cause Retinitis Pigmentosa in humans, a visual disorder characterized by photoreceptors neurodegeneration and progressive vision loss. CERKL produces multiple isoforms with a dynamic subcellular localization. Here we show that a pool of CERKL isoforms localizes at mitochondria in mouse retinal ganglion cells. The depletion of CERKL levels in CerklKD/KO (knockdown/knockout) mouse retinas cause increase of autophagy, mitochondrial fragmentation, alteration of mitochondrial distribution, and dysfunction of mitochondrial-dependent bioenergetics and metabolism. Our results support CERKL as a regulator of autophagy and mitochondrial biology in the mammalian retina. | - |
dc.format.extent | 16 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Elsevier | - |
dc.relation.isformatof | Versió postprint del document publicat a: https://doi.org/10.1016/j.nbd.2021.105405 | - |
dc.relation.ispartof | Neurobiology of Disease, 2021, vol. 156, p. 105405 | - |
dc.relation.uri | https://doi.org/10.1016/j.nbd.2021.105405 | - |
dc.rights | cc-by-nc-nd (c) Elsevier, 2021 | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/4.0/ | - |
dc.source | Articles publicats en revistes (Genètica, Microbiologia i Estadística) | - |
dc.subject.classification | Malalties de la retina | - |
dc.subject.classification | Mitocondris | - |
dc.subject.classification | Mamífers | - |
dc.subject.other | Retinal diseases | - |
dc.subject.other | Mitochondria | - |
dc.subject.other | Mammals | - |
dc.title | CERKL, a retinal dystrophy gene, regulates mitochondrial function and dynamics in the mammalian retina | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/acceptedVersion | - |
dc.identifier.idgrec | 712976 | - |
dc.date.updated | 2021-06-28T12:56:05Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
Appears in Collections: | Articles publicats en revistes (Genètica, Microbiologia i Estadística) |
Files in This Item:
File | Description | Size | Format | |
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712976.pdf | 9.51 MB | Adobe PDF | View/Open |
This item is licensed under a Creative Commons License