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Benedetta Ruzzenente
Researcher at Paris Descartes University
Publications - 38
Citations - 2084
Benedetta Ruzzenente is an academic researcher from Paris Descartes University. The author has contributed to research in topics: Mitochondrial translation & Mitochondrial DNA. The author has an hindex of 19, co-authored 36 publications receiving 1697 citations. Previous affiliations of Benedetta Ruzzenente include University of Paris & Necker-Enfants Malades Hospital.
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Journal ArticleDOI
LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs
Benedetta Ruzzenente,Benedetta Ruzzenente,Metodi D. Metodiev,Anna Wredenberg,Ana Bratic,Ana Bratic,Chan Bae Park,Yolanda Cámara,Dusanka Milenkovic,Volker Zickermann,Rolf Wibom,Kjell Hultenby,Hediye Erdjument-Bromage,Paul Tempst,Ulrich Brandt,James B. Stewart,Claes M. Gustafsson,Nils-Göran Larsson,Nils-Göran Larsson +18 more
TL;DR: It is shown here that the leucine‐rich pentatricopeptide repeat containing (LRPPRC) gene is essential for embryonic development and point to the existence of an elaborate machinery that regulates mammalian mtDNA expression at the post‐transcriptional level.
Journal ArticleDOI
MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome
Yolanda Cámara,Jorge Asin-Cayuela,Chan Bae Park,Metodi D. Metodiev,Yonghong Shi,Yonghong Shi,Benedetta Ruzzenente,Christian Kukat,Bianca Habermann,Rolf Wibom,Kjell Hultenby,Thomas Franz,Hediye Erdjument-Bromage,Paul Tempst,B. Martin Hallberg,Claes M. Gustafsson,Nils-Göran Larsson,Nils-Göran Larsson +17 more
TL;DR: The results thus show that MTERF4 is an important regulator of translation in mammalian mitochondria and directly controls mitochondrial ribosomal biogenesis and translation.
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NSUN4 Is a Dual Function Mitochondrial Protein Required for Both Methylation of 12S rRNA and Coordination of Mitoribosomal Assembly
Metodi D. Metodiev,Henrik Spåhr,Paola Loguercio Polosa,Caroline Meharg,Christian Becker,Janine Altmueller,Bianca Habermann,Nils-Goeran Larsson,Benedetta Ruzzenente +8 more
TL;DR: It is demonstrated that the m5C methyltransferase NSUN4, which forms a complex with MTERF4, is essential in mitochondrial ribosomal biogenesis as mitochondrial translation is abolished in conditional Nsun4 mouse knockouts.
Journal ArticleDOI
TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication
Dusanka Milenkovic,Stanka Matic,Inge Kühl,Benedetta Ruzzenente,Christoph Freyer,Elisabeth Jemt,Chan Bae Park,Maria Falkenberg,Nils-Göran Larsson +8 more
TL;DR: A conditional mouse knockout for Twinkle is presented and it is demonstrated that TWINKLE is essential for mouse embryonic development and thus is the only replicative DNA helicase in mammalian mitochondria, and shown that there is no separateDNA helicase responsible for replication of this region.
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CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels.
Karolina Szczepanowska,Priyanka Maiti,Alexandra Kukat,Eduard Hofsetz,Hendrik Nolte,Katharina Senft,Christina Becker,Benedetta Ruzzenente,Hue-Tran Hornig-Do,Rolf Wibom,Rudolf J. Wiesner,Marcus Krüger,Aleksandra Trifunovic +12 more
TL;DR: Evidence that mammalian CLPP has an essential role in determining the rate of mitochondrial protein synthesis by regulating the level of mitoribosome assembly is presented and the first comprehensive list of possible mammalian ClpXP substrates involved in the regulation of mitochondrial translation, oxidative phosphorylation, and a number of metabolic pathways is produced.