期刊论文详细信息
eLife
Defining the interactome of the human mitochondrial ribosome identifies SMIM4 and TMEM223 as respiratory chain assembly factors
Bettina Warscheid1  Silke Oeljeklaus1  Stefan Stoldt2  Stefan Jakobs3  Johannes Sattmann4  Cong Wang4  Sven Dennerlein4  Elisa Hanitsch4  Sabine Poerschke4  Diana Bauermeister4  Ricarda Richter-Dennerlein5  Peter Rehling6  Thomas Langer7 
[1]Biochemistry and Functional Proteomics, Institute of Biology II, University of Freiburg, Freiburg, Germany
[2]Signalling Research Centres BIOSS and CIBSS, University of Freiburg, Freiburg, Germany
[3]Cluster of Excellence "Multiscale Bioimaging: from Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, Göttingen, Germany
[4]Department of NanoBiophotonics, Max Planck Institute for Biophysical Chemistry, Göttingen, Germany
[5]Department of Neurology, University Medical Center Göttingen, Göttingen, Germany
[6]Cluster of Excellence "Multiscale Bioimaging: from Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, Göttingen, Germany
[7]Department of NanoBiophotonics, Max Planck Institute for Biophysical Chemistry, Göttingen, Germany
[8]Department of Neurology, University Medical Center Göttingen, Göttingen, Germany
[9]Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Translational Neuroinflammation and Automated Microscopy, Göttingen, Germany
[10]Department of Cellular Biochemistry, University Medical Center Göttingen, Göttingen, Germany
[11]Department of Cellular Biochemistry, University Medical Center Göttingen, Göttingen, Germany
[12]Cluster of Excellence "Multiscale Bioimaging: from Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, Göttingen, Germany
[13]Department of Cellular Biochemistry, University Medical Center Göttingen, Göttingen, Germany
[14]Cluster of Excellence "Multiscale Bioimaging: from Molecular Machines to Networks of Excitable Cells" (MBExC), University of Göttingen, Göttingen, Germany
[15]Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Translational Neuroinflammation and Automated Microscopy, Göttingen, Germany
[16]Max Planck Institute for Biophysical Chemistry, Göttingen, Germany
[17]Department of Mitochondrial Proteostasis, Max Planck Institute for Biology of Ageing, Cologne, Germany
关键词: mitochondria;    ribosome;    oxidative phosphorylation;    assembly;    translation;    Mitochondria;   
DOI  :  10.7554/eLife.68213
来源: eLife Sciences Publications, Ltd
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【 摘 要 】
Human mitochondria express a genome that encodes thirteen core subunits of the oxidative phosphorylation system (OXPHOS). These proteins insert into the inner membrane co-translationally. Therefore, mitochondrial ribosomes engage with the OXA1L-insertase and membrane-associated proteins, which support membrane insertion of translation products and early assembly steps into OXPHOS complexes. To identify ribosome-associated biogenesis factors for the OXPHOS system, we purified ribosomes and associated proteins from mitochondria. We identified TMEM223 as a ribosome-associated protein involved in complex IV biogenesis. TMEM223 stimulates the translation of COX1 mRNA and is a constituent of early COX1 assembly intermediates. Moreover, we show that SMIM4 together with C12ORF73 interacts with newly synthesized cytochrome b to support initial steps of complex III biogenesis in complex with UQCC1 and UQCC2. Our analyses define the interactome of the human mitochondrial ribosome and reveal novel assembly factors for complex III and IV biogenesis that link early assembly stages to the translation machinery.
【 授权许可】

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