Defects of mitochondrial RNA turnover lead to the accumulation of double-stranded RNA in vivo

The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully understood. Additionally, upstream processes, such as transcript maturation, have been linked to some of these factors, suggesting either dua...

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Published inPLoS genetics Vol. 15; no. 7; p. e1008240
Main Authors Pajak, Aleksandra, Laine, Isabelle, Clemente, Paula, El-Fissi, Najla, Schober, Florian A., Maffezzini, Camilla, Calvo-Garrido, Javier, Wibom, Rolf, Filograna, Roberta, Dhir, Ashish, Wedell, Anna, Freyer, Christoph, Wredenberg, Anna
Format Journal Article
LanguageEnglish
Published United States Public Library of Science 01.07.2019
Public Library of Science (PLoS)
Subjects
Aging
Animals
Antisense RNA
Biochemistry
Biology
Biology and Life Sciences
Biophysics
CRISPR
Cytoplasm
DEAD-box RNA Helicases - genetics
DEAD-box RNA Helicases - metabolism
Deoxyribonucleic acid
DNA
DNA helicase
DNA-Directed RNA Polymerases - genetics
DNA-Directed RNA Polymerases - metabolism
Double-stranded RNA
Drosophila melanogaster - genetics
Drosophila melanogaster - metabolism
Drosophila Proteins - genetics
Drosophila Proteins - metabolism
Female
Gene expression
Genetic research
Genomes
Helicases
Hospitals
Insects
Laboratories
Male
Mammals
Medicine
Metabolic disorders
Mitochondria
Mitochondrial DNA
Molecular modelling
Molecular structure
mRNA stability
Neoplasm Proteins - genetics
Neoplasm Proteins - metabolism
Phosphorylase
Phosphorylases
Physiology
Poly(A)
Polyadenylation
Polynucleotide adenylyltransferase
Polynucleotide phosphorylase
Polyribonucleotide Nucleotidyltransferase - genetics
Polyribonucleotide Nucleotidyltransferase - metabolism
Research and analysis methods
RNA helicase
RNA interference
RNA Stability
RNA, Antisense - chemistry
RNA, Antisense - metabolism
RNA, Double-Stranded - chemistry
RNA, Double-Stranded - metabolism
RNA, Mitochondrial - chemistry
RNA, Mitochondrial - metabolism
Structure
Supervision
Surgery
Transcription
Sweden
Online AccessGet full text
ISSN1553-7404
1553-7390
1553-7404
DOI10.1371/journal.pgen.1008240

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Abstract The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully understood. Additionally, upstream processes, such as transcript maturation, have been linked to some of these factors, suggesting either dual roles or tightly interconnected mechanisms of mitochondrial RNA metabolism. To get a better understanding of the turn-over of mitochondrial RNAs in vivo, we manipulated the mitochondrial mRNA degrading complex in Drosophila melanogaster models and studied the molecular consequences. Additionally, we investigated if and how these factors interact with the mitochondrial poly(A) polymerase, MTPAP, as well as with the mitochondrial mRNA stabilising factor, LRPPRC. Our results demonstrate a tight interdependency of mitochondrial mRNA stability, polyadenylation and the removal of antisense RNA. Furthermore, disruption of degradation, as well as polyadenylation, leads to the accumulation of double-stranded RNAs, and their escape out into the cytoplasm is associated with an altered immune-response in flies. Together our results suggest a highly organised and inter-dependable regulation of mitochondrial RNA metabolism with far reaching consequences on cellular physiology.
AbstractList The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully understood. Additionally, upstream processes, such as transcript maturation, have been linked to some of these factors, suggesting either dual roles or tightly interconnected mechanisms of mitochondrial RNA metabolism. To get a better understanding of the turn-over of mitochondrial RNAs in vivo, we manipulated the mitochondrial mRNA degrading complex in Drosophila melanogaster models and studied the molecular consequences. Additionally, we investigated if and how these factors interact with the mitochondrial poly(A) polymerase, MTPAP, as well as with the mitochondrial mRNA stabilising factor, LRPPRC. Our results demonstrate a tight interdependency of mitochondrial mRNA stability, polyadenylation and the removal of antisense RNA. Furthermore, disruption of degradation, as well as polyadenylation, leads to the accumulation of double-stranded RNAs, and their escape out into the cytoplasm is associated with an altered immune-response in flies. Together our results suggest a highly organised and inter-dependable regulation of mitochondrial RNA metabolism with far reaching consequences on cellular physiology.
The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully understood. Additionally, upstream processes, such as transcript maturation, have been linked to some of these factors, suggesting either dual roles or tightly interconnected mechanisms of mitochondrial RNA metabolism. To get a better understanding of the turn-over of mitochondrial RNAs in vivo , we manipulated the mitochondrial mRNA degrading complex in Drosophila melanogaster models and studied the molecular consequences. Additionally, we investigated if and how these factors interact with the mitochondrial poly(A) polymerase, MTPAP, as well as with the mitochondrial mRNA stabilising factor, LRPPRC. Our results demonstrate a tight interdependency of mitochondrial mRNA stability, polyadenylation and the removal of antisense RNA. Furthermore, disruption of degradation, as well as polyadenylation, leads to the accumulation of double-stranded RNAs, and their escape out into the cytoplasm is associated with an altered immune-response in flies. Together our results suggest a highly organised and inter-dependable regulation of mitochondrial RNA metabolism with far reaching consequences on cellular physiology. Although a number of factors have been implemented in the turnover of mitochondrial (mt) DNA-derived transcripts, their exact functions and interplay with one another is not entirely clear. Several of these factors have been proposed to co-ordinately regulate both transcript maturation, as well as degradation, but the order of events during mitochondrial RNA turnover is less well understood. Using a range of different genetically modified D rosophila melanogaster models, we studied the involvement of the RNA helicase SUV3, the polynucleotide phosphorylase PNPase, the leucine-rich pentatricopeptide repeat motif-containing protein LRPPRC, and the mitochondrial RNA poly(A) polymerase MTPAP, in stabilisation, polyadenylation, and degradation of mitochondrial transcripts. Our results show a tight collaborative activity of these factors in vivo and reveal a clear hierarchical order of events leading to mitochondrial mRNA maturation. Furthermore, we demonstrate that the loss of SUV3, PNPase, or MTPAP leads to the accumulation of mitochondrial-derived antisense RNA in the cytoplasm of cells, which is associated with an altered immune-response in flies.
The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully understood. Additionally, upstream processes, such as transcript maturation, have been linked to some of these factors, suggesting either dual roles or tightly interconnected mechanisms of mitochondrial RNA metabolism. To get a better understanding of the turn-over of mitochondrial RNAs in vivo, we manipulated the mitochondrial mRNA degrading complex in Drosophila melanogaster models and studied the molecular consequences. Additionally, we investigated if and how these factors interact with the mitochondrial poly(A) polymerase, MTPAP, as well as with the mitochondrial mRNA stabilising factor, LRPPRC. Our results demonstrate a tight interdependency of mitochondrial mRNA stability, polyadenylation and the removal of antisense RNA. Furthermore, disruption of degradation, as well as polyadenylation, leads to the accumulation of double-stranded RNAs, and their escape out into the cytoplasm is associated with an altered immune-response in flies. Together our results suggest a highly organised and inter-dependable regulation of mitochondrial RNA metabolism with far reaching consequences on cellular physiology.The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully understood. Additionally, upstream processes, such as transcript maturation, have been linked to some of these factors, suggesting either dual roles or tightly interconnected mechanisms of mitochondrial RNA metabolism. To get a better understanding of the turn-over of mitochondrial RNAs in vivo, we manipulated the mitochondrial mRNA degrading complex in Drosophila melanogaster models and studied the molecular consequences. Additionally, we investigated if and how these factors interact with the mitochondrial poly(A) polymerase, MTPAP, as well as with the mitochondrial mRNA stabilising factor, LRPPRC. Our results demonstrate a tight interdependency of mitochondrial mRNA stability, polyadenylation and the removal of antisense RNA. Furthermore, disruption of degradation, as well as polyadenylation, leads to the accumulation of double-stranded RNAs, and their escape out into the cytoplasm is associated with an altered immune-response in flies. Together our results suggest a highly organised and inter-dependable regulation of mitochondrial RNA metabolism with far reaching consequences on cellular physiology.
Audience Academic
Author Wredenberg, Anna
El-Fissi, Najla
Wedell, Anna
Pajak, Aleksandra
Calvo-Garrido, Javier
Filograna, Roberta
Maffezzini, Camilla
Wibom, Rolf
Clemente, Paula
Laine, Isabelle
Freyer, Christoph
Dhir, Ashish
Schober, Florian A.
AuthorAffiliation 1 Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden
3 Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
2 Max Planck Institute Biology of Ageing - Karolinska Institutet Laboratory, Karolinska Institutet, Stockholm, Sweden
University of Cologne, GERMANY
4 Centre for Inherited Metabolic Diseases, Karolinska University Hospital, Stockholm, Sweden
5 Centre for Genomic and Experimental Medicine, MRC Institute of Genetics and Molecular Medicine, University of Edinburgh, Edinburgh, United Kingdom
AuthorAffiliation_xml – name: 4 Centre for Inherited Metabolic Diseases, Karolinska University Hospital, Stockholm, Sweden
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– name: University of Cologne, GERMANY
– name: 2 Max Planck Institute Biology of Ageing - Karolinska Institutet Laboratory, Karolinska Institutet, Stockholm, Sweden
– name: 3 Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
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  surname: Wredenberg
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/31365523$$D View this record in MEDLINE/PubMed
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ContentType Journal Article
Copyright COPYRIGHT 2019 Public Library of Science
2019 Pajak et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.
2019 Pajak et al 2019 Pajak et al
Copyright_xml – notice: COPYRIGHT 2019 Public Library of Science
– notice: 2019 Pajak et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.
– notice: 2019 Pajak et al 2019 Pajak et al
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Snippet The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully...
The RNA helicase SUV3 and the polynucleotide phosphorylase PNPase are involved in the degradation of mitochondrial mRNAs but their roles in vivo are not fully...
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StartPage e1008240
SubjectTerms Aging
Animals
Antisense RNA
Biochemistry
Biology
Biology and Life Sciences
Biophysics
CRISPR
Cytoplasm
DEAD-box RNA Helicases - genetics
DEAD-box RNA Helicases - metabolism
Deoxyribonucleic acid
DNA
DNA helicase
DNA-Directed RNA Polymerases - genetics
DNA-Directed RNA Polymerases - metabolism
Double-stranded RNA
Drosophila melanogaster - genetics
Drosophila melanogaster - metabolism
Drosophila Proteins - genetics
Drosophila Proteins - metabolism
Female
Gene expression
Genetic research
Genomes
Helicases
Hospitals
Insects
Laboratories
Male
Mammals
Medicine
Metabolic disorders
Mitochondria
Mitochondrial DNA
Molecular modelling
Molecular structure
mRNA stability
Neoplasm Proteins - genetics
Neoplasm Proteins - metabolism
Phosphorylase
Phosphorylases
Physiology
Poly(A)
Polyadenylation
Polynucleotide adenylyltransferase
Polynucleotide phosphorylase
Polyribonucleotide Nucleotidyltransferase - genetics
Polyribonucleotide Nucleotidyltransferase - metabolism
Research and analysis methods
RNA helicase
RNA interference
RNA Stability
RNA, Antisense - chemistry
RNA, Antisense - metabolism
RNA, Double-Stranded - chemistry
RNA, Double-Stranded - metabolism
RNA, Mitochondrial - chemistry
RNA, Mitochondrial - metabolism
Structure
Supervision
Surgery
Transcription
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Title Defects of mitochondrial RNA turnover lead to the accumulation of double-stranded RNA in vivo
URI https://www.ncbi.nlm.nih.gov/pubmed/31365523
https://www.proquest.com/docview/2274424422
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https://doaj.org/article/ea7ce131776e4971ad749d7f42cbb85b
http://dx.doi.org/10.1371/journal.pgen.1008240
Volume 15
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