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http://hdl.handle.net/2445/206412
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DC Field | Value | Language |
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dc.contributor.author | García-Arroyo, Rocío | - |
dc.contributor.author | Domènech, Elena B. | - |
dc.contributor.author | Herrera Úbeda, Carlos | - |
dc.contributor.author | Asensi, Miguel A. | - |
dc.contributor.author | Núñez de Arenas, Cristina | - |
dc.contributor.author | Cuezva, José M. | - |
dc.contributor.author | Garcia Fernández, Jordi | - |
dc.contributor.author | Pallardó, Federico V. | - |
dc.contributor.author | Mirra, Serena | - |
dc.contributor.author | Marfany i Nadal, Gemma | - |
dc.date.accessioned | 2024-01-26T15:48:47Z | - |
dc.date.available | 2024-01-26T15:48:47Z | - |
dc.date.issued | 2023-10-01 | - |
dc.identifier.issn | 2213-2317 | - |
dc.identifier.uri | http://hdl.handle.net/2445/206412 | - |
dc.description.abstract | The retina is particularly vulnerable to genetic and environmental alterations that generate oxidative stress and cause cellular damage in photoreceptors and other retinal neurons, eventually leading to cell death. <em>CERKL </em>(<em>CERamide Kinase-Like</em>) mutations cause Retinitis Pigmentosa and Cone-Rod Dystrophy in humans, two disorders characterized by photoreceptor degeneration and progressive vision loss. <em>CERKL </em>is a resilience gene against oxidative stress, and its overexpression protects cells from oxidative stress-induced apoptosis. Besides, CERKL contributes to stress granule-formation and regulates mitochondrial dynamics in the retina. Using the <em>CerklKD/KO </em>albino mouse model, which recapitulates the human disease, we aimed to study the impact of <em>Cerkl </em>knockdown on stress response and activation of photoreceptor death mechanisms upon light/oxidative stress. After acute light injury, we assessed immediate or late retinal stress response, by combining both omic and non-omic approaches. Our results show that <em>Cerkl </em>knockdown increases ROS levels and causes a basal exacerbated stress state in the retina, through alterations in glutathione metabolism and stress granule production, overall compromising an adequate response to additional oxidative damage. As a consequence, several cell death mechanisms are triggered in <em>CerklKD/KO </em>retinas after acute light stress. Our studies indicate that <em>Cerkl </em>gene is a pivotal player in regulating light-challenged retinal homeostasis and shed light on how mutations in <em>CERKL </em>lead to blindness by dysregulation of the basal oxidative stress response in the retina. | - |
dc.format.extent | 15 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Elsevier B.V. | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1016/j.redox.2023.102862 | - |
dc.relation.ispartof | Redox Biology, 2023, vol. 66, p. 1-15 | - |
dc.relation.uri | https://doi.org/10.1016/j.redox.2023.102862 | - |
dc.rights | cc-by-nc-nd (c) García-Arroyo, Rocío et al., 2023 | - |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/3.0/es/ | * |
dc.source | Articles publicats en revistes (Genètica, Microbiologia i Estadística) | - |
dc.subject.classification | Malalties de la retina | - |
dc.subject.classification | Estrès oxidatiu | - |
dc.subject.other | Retinal diseases | - |
dc.subject.other | Oxidative stress | - |
dc.title | Exacerbated response to oxidative stress in the Retinitis Pigmentosa CerklKD/KO mouse model triggers retinal degeneration pathways upon acute light stress | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 739390 | - |
dc.date.updated | 2024-01-26T15:48:51Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
Appears in Collections: | Articles publicats en revistes (Genètica, Microbiologia i Estadística) |
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File | Description | Size | Format | |
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828623.pdf | 10.21 MB | Adobe PDF | View/Open |
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