The E3 ubiquitin ligase FBXL6 controls the quality of newly synthesized mitochondrial ribosomal proteins.
| dc.rights.license | open | en_US |
| hal.structure.identifier | Laboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM] | |
| dc.contributor.author | LAVIE, Julie | |
| hal.structure.identifier | Laboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM] | |
| dc.contributor.author | LALOU, Claude | |
| hal.structure.identifier | BoRdeaux Institute in onCology [Inserm U1312 - BRIC] | |
| dc.contributor.author | MAHFOUF, Walid | |
| hal.structure.identifier | Plateforme Protéome [Bordeaux] | |
| dc.contributor.author | DUPUY, Jean-William | |
| hal.structure.identifier | Laboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM] | |
| dc.contributor.author | LACAULE, Aurélie | |
| hal.structure.identifier | Laboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM] | |
| dc.contributor.author | CYWINSKA, Agata Ars | |
| hal.structure.identifier | Laboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM] | |
| dc.contributor.author | LACOMBE, Didier | |
| hal.structure.identifier | Institut de Biologie Moléculaire des Plantes [IBMP] | |
| dc.contributor.author | DUCHENE, Anne-Marie | |
| hal.structure.identifier | BoRdeaux Institute in onCology [Inserm U1312 - BRIC] | |
| dc.contributor.author | RAYMOND, Anne-Aurélie | |
| hal.structure.identifier | BoRdeaux Institute in onCology [Inserm U1312 - BRIC] | |
| dc.contributor.author | REZVANI, Hamid Reza | |
| hal.structure.identifier | Institut de Biologie Moléculaire des Plantes [IBMP] | |
| dc.contributor.author | NGONDO, Richard Patryk | |
| hal.structure.identifier | Laboratoire Maladies Rares: Génétique et Métabolisme (Bordeaux) [U1211 INSERM/MRGM] | |
| dc.contributor.author | BÉNARD, Giovanni | |
| dc.date.accessioned | 2024-02-15T15:58:33Z | |
| dc.date.available | 2024-02-15T15:58:33Z | |
| dc.date.issued | 2023-06-01 | |
| dc.identifier.issn | 2211-1247 | en_US |
| dc.identifier.uri | https://oskar-bordeaux.fr/handle/20.500.12278/188169 | |
| dc.description.abstractEn | In 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.iso | EN | en_US |
| dc.subject.en | Mammals | |
| dc.subject.en | Mitochondria | |
| dc.subject.en | Mitochondrial Proteins | |
| dc.subject.en | Protein Domains | |
| dc.subject.en | Ribosomal Proteins | |
| dc.subject.en | Ubiquitin-Protein Ligases | |
| dc.subject.en | Humans | |
| dc.title.en | The E3 ubiquitin ligase FBXL6 controls the quality of newly synthesized mitochondrial ribosomal proteins. | |
| dc.title.alternative | Cell Rep | en_US |
| dc.type | Article de revue | en_US |
| dc.identifier.doi | 10.1016/j.celrep.2023.112579 | en_US |
| dc.subject.hal | Sciences du Vivant [q-bio]/Biochimie, Biologie Moléculaire | en_US |
| dc.identifier.pubmed | 37267103 | en_US |
| bordeaux.journal | Cell Reports | en_US |
| bordeaux.page | 112579 | en_US |
| bordeaux.volume | 42 | en_US |
| bordeaux.hal.laboratories | Maladies Rares : Génétique et Métabolisme (MRGM) - UMR 1211 | en_US |
| bordeaux.issue | 6 | en_US |
| bordeaux.institution | Université de Bordeaux | en_US |
| bordeaux.institution | INSERM | en_US |
| bordeaux.peerReviewed | oui | en_US |
| bordeaux.inpress | non | en_US |
| bordeaux.import.source | pubmed | |
| hal.popular | non | en_US |
| hal.audience | Internationale | en_US |
| hal.export | false | |
| workflow.import.source | pubmed | |
| dc.rights.cc | Pas de Licence CC | en_US |
| bordeaux.COinS | ctx_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 |
