Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly.
Identifieur interne : 001D65 ( Main/Exploration ); précédent : 001D64; suivant : 001D66Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly.
Auteurs : Oliver Rackham [Australie] ; Jakob D. Busch [Allemagne] ; Stanka Matic [Allemagne] ; Stefan J. Siira [Australie] ; Irina Kuznetsova [Australie] ; Ilian Atanassov [Allemagne] ; Judith A. Ermer [Australie] ; Anne-Marie J. Shearwood [Australie] ; Tara R. Richman [Australie] ; James B. Stewart [Allemagne] ; Arnaud Mourier [France] ; Dusanka Milenkovic [Allemagne] ; Nils-Göran Larsson [Suède] ; Aleksandra Filipovska [Australie]Source :
- Cell reports [ 2211-1247 ] ; 2016.
Descripteurs français
- KwdFr :
- ARN de transfert (génétique), ARN de transfert (métabolisme), ARN ribosomique 16S (génétique), ARN ribosomique 16S (métabolisme), Animaux, Biogenèse des organelles, Biosynthèse des protéines, Cardiomyopathies (anatomopathologie), Cardiomyopathies (génétique), Cardiomyopathies (métabolisme), Fractionnement cellulaire, Maturation post-transcriptionnelle des ARN, Mitochondries du myocarde (génétique), Mitochondries du myocarde (métabolisme), Muscles squelettiques, Myocarde (anatomopathologie), Myocarde (métabolisme), Protéines mitochondriales (génétique), Protéines mitochondriales (métabolisme), Protéines ribosomiques (génétique), Protéines ribosomiques (métabolisme), Ribonuclease P (déficit), Ribonuclease P (génétique), Ribosomes mitochondriaux (métabolisme), Souris, Souris de lignée C57BL, Souris knockout, Transcription génétique, Transcriptome.
- MESH :
- anatomopathologie : Cardiomyopathies, Myocarde.
- déficit : Ribonuclease P.
- génétique : ARN de transfert, ARN ribosomique 16S, Cardiomyopathies, Mitochondries du myocarde, Protéines mitochondriales, Protéines ribosomiques, Ribonuclease P.
- métabolisme : ARN de transfert, ARN ribosomique 16S, Cardiomyopathies, Mitochondries du myocarde, Myocarde, Protéines mitochondriales, Protéines ribosomiques, Ribosomes mitochondriaux.
- Animaux, Biogenèse des organelles, Biosynthèse des protéines, Fractionnement cellulaire, Maturation post-transcriptionnelle des ARN, Muscles squelettiques, Souris, Souris de lignée C57BL, Souris knockout, Transcription génétique, Transcriptome.
English descriptors
- KwdEn :
- Animals, Cardiomyopathies (genetics), Cardiomyopathies (metabolism), Cardiomyopathies (pathology), Cell Fractionation, Mice, Mice, Inbred C57BL, Mice, Knockout, Mitochondria, Heart (genetics), Mitochondria, Heart (metabolism), Mitochondrial Proteins (genetics), Mitochondrial Proteins (metabolism), Mitochondrial Ribosomes (metabolism), Muscle, Skeletal, Myocardium (metabolism), Myocardium (pathology), Organelle Biogenesis, Protein Biosynthesis, RNA Processing, Post-Transcriptional, RNA, Ribosomal, 16S (genetics), RNA, Ribosomal, 16S (metabolism), RNA, Transfer (genetics), RNA, Transfer (metabolism), Ribonuclease P (deficiency), Ribonuclease P (genetics), Ribosomal Proteins (genetics), Ribosomal Proteins (metabolism), Transcription, Genetic, Transcriptome.
- MESH :
- chemical , deficiency : Ribonuclease P.
- chemical , genetics : Mitochondrial Proteins, RNA, Ribosomal, 16S, RNA, Transfer, Ribonuclease P, Ribosomal Proteins.
- genetics : Cardiomyopathies, Mitochondria, Heart.
- metabolism : Cardiomyopathies, Mitochondria, Heart, Mitochondrial Proteins, Mitochondrial Ribosomes, Myocardium, RNA, Ribosomal, 16S, RNA, Transfer, Ribosomal Proteins.
- pathology : Cardiomyopathies, Myocardium.
- Animals, Cell Fractionation, Mice, Mice, Inbred C57BL, Mice, Knockout, Muscle, Skeletal, Organelle Biogenesis, Protein Biosynthesis, RNA Processing, Post-Transcriptional, Transcription, Genetic, Transcriptome.
Abstract
The regulation of mitochondrial RNA processing and its importance for ribosome biogenesis and energy metabolism are not clear. We generated conditional knockout mice of the endoribonuclease component of the RNase P complex, MRPP3, and report that it is essential for life and that heart and skeletal-muscle-specific knockout leads to severe cardiomyopathy, indicating that its activity is non-redundant. Transcriptome-wide parallel analyses of RNA ends (PARE) and RNA-seq enabled us to identify that in vivo 5' tRNA cleavage precedes 3' tRNA processing, and this is required for the correct biogenesis of the mitochondrial ribosomal subunits. We identify that mitoribosomal biogenesis proceeds co-transcriptionally because large mitoribosomal proteins can form a subcomplex on an unprocessed RNA containing the 16S rRNA. Taken together, our data show that RNA processing links transcription to translation via assembly of the mitoribosome.
DOI: 10.1016/j.celrep.2016.07.031
PubMed: 27498866
Affiliations:
- Allemagne, Australie, France, Suède
- District de Cologne, Rhénanie-du-Nord-Westphalie, Svealand
- Cologne, Stockholm
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Le document en format XML
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<author><name sortKey="Siira, Stefan J" sort="Siira, Stefan J" uniqKey="Siira S" first="Stefan J" last="Siira">Stefan J. Siira</name>
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<author><name sortKey="Kuznetsova, Irina" sort="Kuznetsova, Irina" uniqKey="Kuznetsova I" first="Irina" last="Kuznetsova">Irina Kuznetsova</name>
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<author><name sortKey="Atanassov, Ilian" sort="Atanassov, Ilian" uniqKey="Atanassov I" first="Ilian" last="Atanassov">Ilian Atanassov</name>
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<author><name sortKey="Ermer, Judith A" sort="Ermer, Judith A" uniqKey="Ermer J" first="Judith A" last="Ermer">Judith A. Ermer</name>
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<author><name sortKey="Richman, Tara R" sort="Richman, Tara R" uniqKey="Richman T" first="Tara R" last="Richman">Tara R. Richman</name>
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<author><name sortKey="Stewart, James B" sort="Stewart, James B" uniqKey="Stewart J" first="James B" last="Stewart">James B. Stewart</name>
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<country xml:lang="fr">Suède</country>
<wicri:regionArea>Department of Mitochondrial Biology, Max Planck Institute for Biology of Ageing, 50931 Cologne, Germany; Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm 17177</wicri:regionArea>
<placeName><settlement type="city">Stockholm</settlement>
<region nuts="2">Svealand</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Filipovska, Aleksandra" sort="Filipovska, Aleksandra" uniqKey="Filipovska A" first="Aleksandra" last="Filipovska">Aleksandra Filipovska</name>
<affiliation wicri:level="1"><nlm:affiliation>Harry Perkins Institute of Medical Research and Centre for Medical Research, The University of Western Australia, Nedlands, WA 6009, Australia; School of Chemistry and Biochemistry, The University of Western Australia, Nedlands, WA 6009, Australia. Electronic address: aleksandra.filipovska@uwa.edu.au.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Harry Perkins Institute of Medical Research and Centre for Medical Research, The University of Western Australia, Nedlands, WA 6009, Australia; School of Chemistry and Biochemistry, The University of Western Australia, Nedlands, WA 6009</wicri:regionArea>
<wicri:noRegion>WA 6009</wicri:noRegion>
</affiliation>
</author>
</analytic>
<series><title level="j">Cell reports</title>
<idno type="eISSN">2211-1247</idno>
<imprint><date when="2016" type="published">2016</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Animals</term>
<term>Cardiomyopathies (genetics)</term>
<term>Cardiomyopathies (metabolism)</term>
<term>Cardiomyopathies (pathology)</term>
<term>Cell Fractionation</term>
<term>Mice</term>
<term>Mice, Inbred C57BL</term>
<term>Mice, Knockout</term>
<term>Mitochondria, Heart (genetics)</term>
<term>Mitochondria, Heart (metabolism)</term>
<term>Mitochondrial Proteins (genetics)</term>
<term>Mitochondrial Proteins (metabolism)</term>
<term>Mitochondrial Ribosomes (metabolism)</term>
<term>Muscle, Skeletal</term>
<term>Myocardium (metabolism)</term>
<term>Myocardium (pathology)</term>
<term>Organelle Biogenesis</term>
<term>Protein Biosynthesis</term>
<term>RNA Processing, Post-Transcriptional</term>
<term>RNA, Ribosomal, 16S (genetics)</term>
<term>RNA, Ribosomal, 16S (metabolism)</term>
<term>RNA, Transfer (genetics)</term>
<term>RNA, Transfer (metabolism)</term>
<term>Ribonuclease P (deficiency)</term>
<term>Ribonuclease P (genetics)</term>
<term>Ribosomal Proteins (genetics)</term>
<term>Ribosomal Proteins (metabolism)</term>
<term>Transcription, Genetic</term>
<term>Transcriptome</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>ARN de transfert (génétique)</term>
<term>ARN de transfert (métabolisme)</term>
<term>ARN ribosomique 16S (génétique)</term>
<term>ARN ribosomique 16S (métabolisme)</term>
<term>Animaux</term>
<term>Biogenèse des organelles</term>
<term>Biosynthèse des protéines</term>
<term>Cardiomyopathies (anatomopathologie)</term>
<term>Cardiomyopathies (génétique)</term>
<term>Cardiomyopathies (métabolisme)</term>
<term>Fractionnement cellulaire</term>
<term>Maturation post-transcriptionnelle des ARN</term>
<term>Mitochondries du myocarde (génétique)</term>
<term>Mitochondries du myocarde (métabolisme)</term>
<term>Muscles squelettiques</term>
<term>Myocarde (anatomopathologie)</term>
<term>Myocarde (métabolisme)</term>
<term>Protéines mitochondriales (génétique)</term>
<term>Protéines mitochondriales (métabolisme)</term>
<term>Protéines ribosomiques (génétique)</term>
<term>Protéines ribosomiques (métabolisme)</term>
<term>Ribonuclease P (déficit)</term>
<term>Ribonuclease P (génétique)</term>
<term>Ribosomes mitochondriaux (métabolisme)</term>
<term>Souris</term>
<term>Souris de lignée C57BL</term>
<term>Souris knockout</term>
<term>Transcription génétique</term>
<term>Transcriptome</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="deficiency" xml:lang="en"><term>Ribonuclease P</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Mitochondrial Proteins</term>
<term>RNA, Ribosomal, 16S</term>
<term>RNA, Transfer</term>
<term>Ribonuclease P</term>
<term>Ribosomal Proteins</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Cardiomyopathies</term>
<term>Myocarde</term>
</keywords>
<keywords scheme="MESH" qualifier="déficit" xml:lang="fr"><term>Ribonuclease P</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Cardiomyopathies</term>
<term>Mitochondria, Heart</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>ARN de transfert</term>
<term>ARN ribosomique 16S</term>
<term>Cardiomyopathies</term>
<term>Mitochondries du myocarde</term>
<term>Protéines mitochondriales</term>
<term>Protéines ribosomiques</term>
<term>Ribonuclease P</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Cardiomyopathies</term>
<term>Mitochondria, Heart</term>
<term>Mitochondrial Proteins</term>
<term>Mitochondrial Ribosomes</term>
<term>Myocardium</term>
<term>RNA, Ribosomal, 16S</term>
<term>RNA, Transfer</term>
<term>Ribosomal Proteins</term>
</keywords>
<keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>ARN de transfert</term>
<term>ARN ribosomique 16S</term>
<term>Cardiomyopathies</term>
<term>Mitochondries du myocarde</term>
<term>Myocarde</term>
<term>Protéines mitochondriales</term>
<term>Protéines ribosomiques</term>
<term>Ribosomes mitochondriaux</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Cardiomyopathies</term>
<term>Myocardium</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Animals</term>
<term>Cell Fractionation</term>
<term>Mice</term>
<term>Mice, Inbred C57BL</term>
<term>Mice, Knockout</term>
<term>Muscle, Skeletal</term>
<term>Organelle Biogenesis</term>
<term>Protein Biosynthesis</term>
<term>RNA Processing, Post-Transcriptional</term>
<term>Transcription, Genetic</term>
<term>Transcriptome</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Animaux</term>
<term>Biogenèse des organelles</term>
<term>Biosynthèse des protéines</term>
<term>Fractionnement cellulaire</term>
<term>Maturation post-transcriptionnelle des ARN</term>
<term>Muscles squelettiques</term>
<term>Souris</term>
<term>Souris de lignée C57BL</term>
<term>Souris knockout</term>
<term>Transcription génétique</term>
<term>Transcriptome</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">The regulation of mitochondrial RNA processing and its importance for ribosome biogenesis and energy metabolism are not clear. We generated conditional knockout mice of the endoribonuclease component of the RNase P complex, MRPP3, and report that it is essential for life and that heart and skeletal-muscle-specific knockout leads to severe cardiomyopathy, indicating that its activity is non-redundant. Transcriptome-wide parallel analyses of RNA ends (PARE) and RNA-seq enabled us to identify that in vivo 5' tRNA cleavage precedes 3' tRNA processing, and this is required for the correct biogenesis of the mitochondrial ribosomal subunits. We identify that mitoribosomal biogenesis proceeds co-transcriptionally because large mitoribosomal proteins can form a subcomplex on an unprocessed RNA containing the 16S rRNA. Taken together, our data show that RNA processing links transcription to translation via assembly of the mitoribosome.</div>
</front>
</TEI>
<affiliations><list><country><li>Allemagne</li>
<li>Australie</li>
<li>France</li>
<li>Suède</li>
</country>
<region><li>District de Cologne</li>
<li>Rhénanie-du-Nord-Westphalie</li>
<li>Svealand</li>
</region>
<settlement><li>Cologne</li>
<li>Stockholm</li>
</settlement>
</list>
<tree><country name="Australie"><noRegion><name sortKey="Rackham, Oliver" sort="Rackham, Oliver" uniqKey="Rackham O" first="Oliver" last="Rackham">Oliver Rackham</name>
</noRegion>
<name sortKey="Ermer, Judith A" sort="Ermer, Judith A" uniqKey="Ermer J" first="Judith A" last="Ermer">Judith A. Ermer</name>
<name sortKey="Filipovska, Aleksandra" sort="Filipovska, Aleksandra" uniqKey="Filipovska A" first="Aleksandra" last="Filipovska">Aleksandra Filipovska</name>
<name sortKey="Kuznetsova, Irina" sort="Kuznetsova, Irina" uniqKey="Kuznetsova I" first="Irina" last="Kuznetsova">Irina Kuznetsova</name>
<name sortKey="Richman, Tara R" sort="Richman, Tara R" uniqKey="Richman T" first="Tara R" last="Richman">Tara R. Richman</name>
<name sortKey="Shearwood, Anne Marie J" sort="Shearwood, Anne Marie J" uniqKey="Shearwood A" first="Anne-Marie J" last="Shearwood">Anne-Marie J. Shearwood</name>
<name sortKey="Siira, Stefan J" sort="Siira, Stefan J" uniqKey="Siira S" first="Stefan J" last="Siira">Stefan J. Siira</name>
</country>
<country name="Allemagne"><region name="Rhénanie-du-Nord-Westphalie"><name sortKey="Busch, Jakob D" sort="Busch, Jakob D" uniqKey="Busch J" first="Jakob D" last="Busch">Jakob D. Busch</name>
</region>
<name sortKey="Atanassov, Ilian" sort="Atanassov, Ilian" uniqKey="Atanassov I" first="Ilian" last="Atanassov">Ilian Atanassov</name>
<name sortKey="Matic, Stanka" sort="Matic, Stanka" uniqKey="Matic S" first="Stanka" last="Matic">Stanka Matic</name>
<name sortKey="Milenkovic, Dusanka" sort="Milenkovic, Dusanka" uniqKey="Milenkovic D" first="Dusanka" last="Milenkovic">Dusanka Milenkovic</name>
<name sortKey="Stewart, James B" sort="Stewart, James B" uniqKey="Stewart J" first="James B" last="Stewart">James B. Stewart</name>
</country>
<country name="France"><noRegion><name sortKey="Mourier, Arnaud" sort="Mourier, Arnaud" uniqKey="Mourier A" first="Arnaud" last="Mourier">Arnaud Mourier</name>
</noRegion>
</country>
<country name="Suède"><region name="Svealand"><name sortKey="Larsson, Nils Goran" sort="Larsson, Nils Goran" uniqKey="Larsson N" first="Nils-Göran" last="Larsson">Nils-Göran Larsson</name>
</region>
</country>
</tree>
</affiliations>
</record>
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