Dual function of GTPBP6 in biogenesis and recycling of human mitochondrial ribosomes.

Lavdovskaia, Elena; Denks, Kärt; Nadler, Franziska; Steube, Emely; Linden, Andreas; Urlaub, Henning; Rodnina, Marina V; Richter-Dennerlein, Ricarda

Lavdovskaia, Elena; Denks, Kärt; Nadler, Franziska; Steube, Emely; Linden, Andreas; Urlaub, Henning; Rodnina, Marina V; Richter-Dennerlein, Ricarda.

Afiliación

Lavdovskaia E; Department of Cellular Biochemistry, University Medical Center Goettingen, D-37073 Goettingen, Germany.
Denks K; Cluster of Excellence 'Multiscale Bioimaging: from Molecular Machines to Networks of Excitable Cells' (MBExC), University of Goettingen, Goettingen, Germany.
Nadler F; Cluster of Excellence 'Multiscale Bioimaging: from Molecular Machines to Networks of Excitable Cells' (MBExC), University of Goettingen, Goettingen, Germany.
Steube E; Department of Physical Biochemistry, Max Planck Institute for Biophysical Chemistry, D-37077 Goettingen, Germany.
Linden A; Department of Cellular Biochemistry, University Medical Center Goettingen, D-37073 Goettingen, Germany.
Urlaub H; Department of Cellular Biochemistry, University Medical Center Goettingen, D-37073 Goettingen, Germany.
Rodnina MV; Bioanalytical Mass Spectrometry Group, Max Planck Institute for Biophysical Chemistry, D-37077 Goettingen, Germany.
Richter-Dennerlein R; Bioanalytics, Institute for Clinical Chemistry, University Medical Center Goettingen, D-37073 Goettingen, Germany.

Nucleic Acids Res ; 48(22): 12929-12942, 2020 12 16.

Article en En | MEDLINE | ID: mdl-33264405

RESUMEN

Translation and ribosome biogenesis in mitochondria require auxiliary factors that ensure rapid and accurate synthesis of mitochondrial proteins. Defects in translation are associated with oxidative phosphorylation deficiency and cause severe human diseases, but the exact roles of mitochondrial translation-associated factors are not known. Here we identify the functions of GTPBP6, a homolog of the bacterial ribosome-recycling factor HflX, in human mitochondria. Similarly to HflX, GTPBP6 facilitates the dissociation of ribosomes in vitro and in vivo. In contrast to HflX, GTPBP6 is also required for the assembly of mitochondrial ribosomes. GTPBP6 ablation leads to accumulation of late assembly intermediate(s) of the large ribosomal subunit containing ribosome biogenesis factors MTERF4, NSUN4, MALSU1 and the GTPases GTPBP5, GTPBP7 and GTPBP10. Our data show that GTPBP6 has a dual function acting in ribosome recycling and biogenesis. These findings contribute to our understanding of large ribosomal subunit assembly as well as ribosome recycling pathway in mitochondria.

Asunto(s)

Proteínas de Unión al GTP/genética; Mitocondrias/genética; Proteínas Mitocondriales/genética; Ribosomas Mitocondriales; GTP Fosfohidrolasas/genética; Humanos; Metiltransferasas/genética; Proteínas de Unión al GTP Monoméricas/genética; Biosíntesis de Proteínas/genética; Proteínas Ribosómicas/genética; Factores de Transcripción/genética

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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Proteínas de Unión al GTP / Proteínas Mitocondriales / Ribosomas Mitocondriales / Mitocondrias Tipo de estudio: Prognostic_studies Límite: Humans Idioma: En Revista: Nucleic Acids Res Año: 2020 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Reino Unido

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