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

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Version: Final published version
License: CC BY 4.0
Serval ID
serval:BIB_95EDC7CF1789
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules.
Journal
The Journal of biological chemistry
Author(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
Publication state
Published
Issued date
17/03/2017
Peer-reviewed
Oui
Volume
292
Number
11
Pages
4519-4532
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
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.
Keywords
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
Yes
Create date
13/04/2021 17:25
Last modification date
08/02/2022 7:36
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