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The pseudouridine synthase RPUSD4 is an essential component of mitochondrial RNA granules

Lookup NU author(s): Dr Agata Rozanska, Professor Robert Lightowlers, Professor Zofia Chrzanowska-Lightowlers

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This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


Abstract

© 2017 by The American Society for Biochemistry and Molecular Biology, Inc. 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, mttRNAMet, 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.


Publication metadata

Author(s): Zaganelli S, Rebelo-Guiomar P, Maundrell K, Rozanska A, Pierredon S, Powell CA, Jourdain AA, Hulo N, Lightowlers RN, Chrzanowska-Lightowlers ZM, Minczuk M, Martinou J-C

Publication type: Article

Publication status: Published

Journal: Journal of Biological Chemistry

Year: 2017

Volume: 292

Issue: 11

Pages: 4519-4532

Print publication date: 17/03/2017

Online publication date: 12/01/2017

Acceptance date: 11/01/2017

Date deposited: 27/04/2017

ISSN (print): 0021-9258

ISSN (electronic): 1083-351X

Publisher: American Society for Biochemistry and Molecular Biology Inc.

URL: https://doi.org/10.1074/jbc.M116.771105

DOI: 10.1074/jbc.M116.771105


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