Please use this identifier to cite or link to this item: http://hdl.handle.net/2445/178699
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dc.contributor.authorMirra, Serena-
dc.contributor.authorGarcía-Arroyo, Rocío-
dc.contributor.authorDomènech, Elena B.-
dc.contributor.authorGavaldà i Navarro, Aleix-
dc.contributor.authorHerrera Úbeda, Carlos-
dc.contributor.authorOliva, Clara-
dc.contributor.authorGarcia Fernández, Jordi-
dc.contributor.authorArtuch Iriberri, Rafael-
dc.contributor.authorVillarroya i Gombau, Francesc-
dc.contributor.authorMarfany i Nadal, Gemma-
dc.date.accessioned2021-06-28T12:56:05Z-
dc.date.available2022-05-25T05:10:21Z-
dc.date.issued2021-05-25-
dc.identifier.issn0969-9961-
dc.identifier.urihttp://hdl.handle.net/2445/178699-
dc.description.abstractThe 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.extent16 p.-
dc.format.mimetypeapplication/pdf-
dc.language.isoeng-
dc.publisherElsevier-
dc.relation.isformatofVersió postprint del document publicat a: https://doi.org/10.1016/j.nbd.2021.105405-
dc.relation.ispartofNeurobiology of Disease, 2021, vol. 156, p. 105405-
dc.relation.urihttps://doi.org/10.1016/j.nbd.2021.105405-
dc.rightscc-by-nc-nd (c) Elsevier, 2021-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/4.0/-
dc.subject.classificationMalalties de la retina-
dc.subject.classificationMitocondris-
dc.subject.classificationMamífers-
dc.subject.otherRetinal diseases-
dc.subject.otherMitochondria-
dc.subject.otherMammals-
dc.titleCERKL, a retinal dystrophy gene, regulates mitochondrial function and dynamics in the mammalian retina-
dc.typeinfo:eu-repo/semantics/article-
dc.typeinfo:eu-repo/semantics/acceptedVersion-
dc.identifier.idgrec712976-
dc.date.updated2021-06-28T12:56:05Z-
dc.rights.accessRightsinfo:eu-repo/semantics/openAccess-
Appears in Collections:Articles publicats en revistes (Genètica, Microbiologia i Estadística)

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