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Translation in Mitochondria and Other Organelles pp 85–107Cite as

Mitochondrial Targeting of RNA and Mitochondrial Translation

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Abstract

Mitochondrial translation depends on the macromolecular components imported from the cytosol, which include translation factors, ribosomal proteins, aminoacyl-tRNA synthetases, and a variable number of small noncoding RNAs. The lasts are essentially tRNAs, but other small RNAs, like mammalian 5S rRNA, are also concerned by the RNA mitochondrial targeting pathway. If their importance in mitochondrial translation was demonstrated in each case where it was addressed, the precise function of these molecules differs from one system to another: in many cases they complement lacking mtDNA encoded counterparts, in others can fulfill conditional functions, finally they can complement the lack of needed mitochondrial enzymatic activities. In any case, it appears that the innated capacity of mitochondria to import small RNA molecules is supplied by specific additional protein, often performing their “second job” to deliver the needed RNA in the organelle. This mechanism, still not understood in details, remains the unique natural pathway of nucleic acids delivery in mitochondria, and is therefore of a significant interest as a tool permitting to target this organelle with potentially therapeutic molecules and thus addressing a very important bulk of human pathologies linked with dysfunctions of mitochondrial translation machinery.

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Acknowledgments

The authors thank all the members of the UMR 7156 GMGM mitochondrial team (Strasbourg, France), H. Becker (Strasbourg, France), R. N. Lightowlers (Newcastle, U.K.), and J. Herrmann (Kaiserslautern, Germany) for helpful discussions. The studies of our team cited in the chapter were supported by the CNRS, University of Strasbourg, ANR (Agence Nationale de Recherche), AFM (Association Française cotre les Myopathies), FRM (Fondation de Recherche Médicale), and the National Program Investissement d’Avenir (LabEx MitoCross).

IC and PK are supported by Russian Foundation for Basic Research and Russian Ministry of Education and Science (Federal programme "Scientific stuff of innovative Russia").

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Authors and Affiliations

  1. Molecular Genetics, Genomics, Microbiology (UMR 7156), Universty of Strasbourg and CNRS, 21 René Descartes, 67084, Strsbourg, France

    Ivan Tarassov, Ivan Chicherin, Yann Tonin, Alexandre Smirnov, Petr Kamenski & Nina Entelis

  2. Molecular Biology Department, Biology Faculty of M. V., Lomonossov Moscow State University, 119991, Moscow, Russia

    Ivan Chicherin, Alexandre Smirnov & Petr Kamenski

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  1. Ivan Tarassov
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  2. Ivan Chicherin
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  3. Yann Tonin
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  4. Alexandre Smirnov
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  5. Petr Kamenski
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  6. Nina Entelis
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Correspondence to Ivan Tarassov .

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  1. Institut de Biologie Moleculaire des Plantes, Strasbourg CX, France

    Anne-Marie Duchêne

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Tarassov, I., Chicherin, I., Tonin, Y., Smirnov, A., Kamenski, P., Entelis, N. (2013). Mitochondrial Targeting of RNA and Mitochondrial Translation. In: Duchêne, AM. (eds) Translation in Mitochondria and Other Organelles. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-39426-3_4

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