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

Détails

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Etat: Public
Version: Final published version
Licence: CC BY 4.0
ID Serval
serval:BIB_95EDC7CF1789
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules.
Périodique
The Journal of biological chemistry
Auteur⸱e⸱s
Zaganelli S., Rebelo-Guiomar P., Maundrell K., Rozanska A., Pierredon S., Powell C.A., Jourdain A.A., Hulo N., Lightowlers R.N., Chrzanowska-Lightowlers Z.M., Minczuk M., Martinou J.C.
ISSN
1083-351X (Electronic)
ISSN-L
0021-9258
Statut éditorial
Publié
Date de publication
17/03/2017
Peer-reviewed
Oui
Volume
292
Numéro
11
Pages
4519-4532
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: ppublish
Résumé
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-tRNA <sup>Met</sup> , and mt-tRNA <sup>Phe</sup> , 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.
Mots-clé
Cell Line, Humans, Intramolecular Transferases/analysis, Intramolecular Transferases/genetics, Intramolecular Transferases/metabolism, Mitochondria/genetics, Mitochondria/metabolism, Mitochondrial Proteins/metabolism, Protein Transport, RNA/metabolism, RNA Interference, RNA, Mitochondrial, RNA, Ribosomal, 16S/metabolism, RNA, Small Interfering/genetics, RNA, Transfer, Met/metabolism, RNA, Transfer, Phe/metabolism, OXPHOS, RNA, RNA modification, RNA-binding protein, RPUSD, mitochondria, mitoribosome biogenesis, pseudouridine, ribosome
Pubmed
Web of science
Open Access
Oui
Création de la notice
13/04/2021 17:25
Dernière modification de la notice
08/02/2022 7:36
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