共查询到20条相似文献,搜索用时 187 毫秒
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Anna Wredenberg Marie Lagouge Ana Bratic Metodi D. Metodiev Henrik Sp?hr Arnaud Mourier Christoph Freyer Benedetta Ruzzenente Luke Tain Sebastian Gr?nke Francesca Baggio Christian Kukat Elisabeth Kremmer Rolf Wibom Paola Loguercio Polosa Bianca Habermann Linda Partridge Chan Bae Park Nils-G?ran Larsson 《PLoS genetics》2013,9(1)
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Metodi Dimitrov Metodiev Henrik Sp?hr Paola Loguercio Polosa Caroline Meharg Christian Becker Janine Altmueller Bianca Habermann Nils-G?ran Larsson Benedetta Ruzzenente 《PLoS genetics》2014,10(2)
Biogenesis of mammalian mitochondrial ribosomes requires a concerted maturation of both the small (SSU) and large subunit (LSU). We demonstrate here 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. Deep sequencing of bisulfite-treated RNA shows that NSUN4 methylates cytosine 911 in 12S rRNA (m5C911) of the SSU. Surprisingly, NSUN4 does not need MTERF4 to generate this modification. Instead, the NSUN4/MTERF4 complex is required to assemble the SSU and LSU to form a monosome. NSUN4 is thus a dual function protein, which on the one hand is needed for 12S rRNA methylation and, on the other hand interacts with MTERF4 to facilitate monosome assembly. The presented data suggest that NSUN4 has a key role in controlling a final step in ribosome biogenesis to ensure that only the mature SSU and LSU are assembled. 相似文献
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Elena Lavdovskaia Krt Denks Franziska Nadler Emely Steube Andreas Linden Henning Urlaub Marina V Rodnina Ricarda Richter-Dennerlein 《Nucleic acids research》2020,48(22):12929
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. 相似文献
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Mitochondrial DNA transcription: regulating the power supply 总被引:1,自引:0,他引:1
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