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  1. AU="Agata Ars Cywinska"
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Artikel ; Online: The E3 ubiquitin ligase FBXL6 controls the quality of newly synthesized mitochondrial ribosomal proteins

Julie Lavie / Claude Lalou / Walid Mahfouf / Jean-William Dupuy / Aurélie Lacaule / Agata Ars Cywinska / Didier Lacombe / Anne-Marie Duchêne / Anne-Aurélie Raymond / Hamid Reza Rezvani / Richard Patryk Ngondo / Giovanni Bénard

Cell Reports, Vol 42, Iss 6, Pp 112579- (2023)

2023  

Abstract: Summary: 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. ...

Abstract Summary: 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.
Schlagwörter CP: Cell biology ; Biology (General) ; QH301-705.5
Thema/Rubrik (Code) 570
Sprache Englisch
Erscheinungsdatum 2023-06-01T00:00:00Z
Verlag Elsevier
Dokumenttyp Artikel ; Online
Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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