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The E3 ubiquitin ligase FBXL6 controls the quality of newly synthesized mitochondrial ribosomal proteins.

dc.rights.licenseopenen_US
hal.structure.identifierLaboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM]
dc.contributor.authorLAVIE, Julie
hal.structure.identifierLaboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM]
dc.contributor.authorLALOU, Claude
hal.structure.identifierBoRdeaux Institute in onCology [Inserm U1312 - BRIC]
dc.contributor.authorMAHFOUF, Walid
hal.structure.identifierPlateforme Protéome [Bordeaux]
dc.contributor.authorDUPUY, Jean-William
hal.structure.identifierLaboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM]
dc.contributor.authorLACAULE, Aurélie
hal.structure.identifierLaboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM]
dc.contributor.authorCYWINSKA, Agata Ars
hal.structure.identifierLaboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM]
dc.contributor.authorLACOMBE, Didier
hal.structure.identifierInstitut de Biologie Moléculaire des Plantes [IBMP]
dc.contributor.authorDUCHENE, Anne-Marie
hal.structure.identifierBoRdeaux Institute in onCology [Inserm U1312 - BRIC]
dc.contributor.authorRAYMOND, Anne-Aurélie
hal.structure.identifierBoRdeaux Institute in onCology [Inserm U1312 - BRIC]
dc.contributor.authorREZVANI, Hamid Reza
hal.structure.identifierInstitut de Biologie Moléculaire des Plantes [IBMP]
dc.contributor.authorNGONDO, Richard Patryk
hal.structure.identifierLaboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM]
dc.contributor.authorBÉNARD, Giovanni
dc.date.accessioned2024-02-15T15:58:33Z
dc.date.available2024-02-15T15:58:33Z
dc.date.issued2023-06-01
dc.identifier.issn2211-1247en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/188169
dc.description.abstractEnIn mammals, about 99% of mitochondrial proteins are synthesized in the cytosol as precursors that are subsequently imported into the organelle. The mitochondrial health and functions rely on an accurate quality control of these imported proteins. Here, we show that the E3 ubiquitin ligase F box/leucine-rich-repeat protein 6 (FBXL6) regulates the quality of cytosolically translated mitochondrial proteins. Indeed, we found that FBXL6 substrates are newly synthesized mitochondrial ribosomal proteins. This E3 binds to chaperones involved in the folding and trafficking of newly synthesized peptide and to ribosomal-associated quality control proteins. Deletion of these interacting partners is sufficient to hamper interactions between FBXL6 and its substrate. Furthermore, we show that cells lacking FBXL6 fail to degrade specifically mistranslated mitochondrial ribosomal proteins. Finally, showing the role of FBXL6-dependent mechanism, FBXL6-knockout (KO) cells display mitochondrial ribosomal protein aggregations, altered mitochondrial metabolism, and inhibited cell cycle in oxidative conditions.
dc.language.isoENen_US
dc.subject.enMammals
dc.subject.enMitochondria
dc.subject.enMitochondrial Proteins
dc.subject.enProtein Domains
dc.subject.enRibosomal Proteins
dc.subject.enUbiquitin-Protein Ligases
dc.subject.enHumans
dc.title.enThe E3 ubiquitin ligase FBXL6 controls the quality of newly synthesized mitochondrial ribosomal proteins.
dc.title.alternativeCell Repen_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1016/j.celrep.2023.112579en_US
dc.subject.halSciences du Vivant [q-bio]/Biochimie, Biologie Moléculaireen_US
dc.identifier.pubmed37267103en_US
bordeaux.journalCell Reportsen_US
bordeaux.page112579en_US
bordeaux.volume42en_US
bordeaux.hal.laboratoriesMaladies Rares : Génétique et Métabolisme (MRGM) - UMR 1211en_US
bordeaux.issue6en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.institutionINSERMen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
bordeaux.import.sourcepubmed
hal.popularnonen_US
hal.audienceInternationaleen_US
hal.exportfalse
workflow.import.sourcepubmed
dc.rights.ccPas de Licence CCen_US
bordeaux.COinSctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Cell%20Reports&rft.date=2023-06-01&rft.volume=42&rft.issue=6&rft.spage=112579&rft.epage=112579&rft.eissn=2211-1247&rft.issn=2211-1247&rft.au=LAVIE,%20Julie&LALOU,%20Claude&MAHFOUF,%20Walid&DUPUY,%20Jean-William&LACAULE,%20Aur%C3%A9lie&rft.genre=article


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