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The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules.

  • Zaganelli S From the Department of Cell Biology, University of Geneva, 30 quai Ernest-Ansermet, 1211 Genève 4, Switzerland.
  • Rebelo-Guiomar P Medical Research Council Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, United Kingdom.
  • Maundrell K From the Department of Cell Biology, University of Geneva, 30 quai Ernest-Ansermet, 1211 Genève 4, Switzerland.
  • Rozanska A Wellcome Trust Centre for Mitochondrial Research, Institute of Cell and Molecular Biosciences, The Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, United Kingdom, and.
  • Pierredon S From the Department of Cell Biology, University of Geneva, 30 quai Ernest-Ansermet, 1211 Genève 4, Switzerland.
  • Powell CA Medical Research Council Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, United Kingdom.
  • Jourdain AA From the Department of Cell Biology, University of Geneva, 30 quai Ernest-Ansermet, 1211 Genève 4, Switzerland.
  • Hulo N Institute of Genetics and Genomics of Geneva, Université de Genève, 1211 Genève 4, Switzerland.
  • Lightowlers RN Wellcome Trust Centre for Mitochondrial Research, Institute of Cell and Molecular Biosciences, The Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, United Kingdom, and.
  • Chrzanowska-Lightowlers ZM Wellcome Trust Centre for Mitochondrial Research, Institute of Cell and Molecular Biosciences, The Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, United Kingdom, and.
  • Minczuk M Medical Research Council Mitochondrial Biology Unit, Hills Road, Cambridge CB2 0XY, United Kingdom.
  • Martinou JC From the Department of Cell Biology, University of Geneva, 30 quai Ernest-Ansermet, 1211 Genève 4, Switzerland, jean-claude.martinou@unige.ch.
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  • 2017-01-14
Published in:
  • The Journal of biological chemistry. - 2017
OXPHOS
RNA
RNA modification
RNA-binding protein
RPUSD
mitochondria
mitoribosome biogenesis
pseudouridine
ribosome
Cell Line
Humans
Intramolecular Transferases
Mitochondria
Mitochondrial Proteins
Protein Transport
RNA
RNA Interference
RNA, Mitochondrial
RNA, Ribosomal, 16S
RNA, Small Interfering
RNA, Transfer, Met
RNA, Transfer, Phe
English Mitochondrial gene expression is a fundamental process that is largely dependent on nuclear-encoded proteins. Several steps of mitochondrial RNA processing and maturation, including RNA post-transcriptional modification, appear to be spatially organized into distinct foci, which we have previously termed mitochondrial RNA granules (MRGs). Although an increasing number of proteins have been localized to MRGs, a comprehensive analysis of the proteome of these structures is still lacking. Here, we have applied a microscopy-based approach that has allowed us to identify novel components of the MRG proteome. Among these, we have focused our attention on RPUSD4, an uncharacterized mitochondrial putative pseudouridine synthase. We show that RPUSD4 depletion leads to a severe reduction of the steady-state level of the 16S mitochondrial (mt) rRNA with defects in the biogenesis of the mitoribosome large subunit and consequently in mitochondrial translation. We report that RPUSD4 binds 16S mt-rRNA, mt-tRNAMet, and mt-tRNAPhe, and we demonstrate that it is responsible for pseudouridylation of the latter. These data provide new insights into the relevance of RNA pseudouridylation in mitochondrial gene expression.
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  • English
Open access status
hybrid
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https://sonar.ch/global/documents/76492
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