Alterations at the Cross-Bridge Level Are Associated with a Paradoxical Gain of Muscle Function In Vivo in a Mouse Model of Nemaline Myopathy
Identifieur interne : 001756 ( Pmc/Checkpoint ); précédent : 001755; suivant : 001757Alterations at the Cross-Bridge Level Are Associated with a Paradoxical Gain of Muscle Function In Vivo in a Mouse Model of Nemaline Myopathy
Auteurs : Charlotte Gineste [France] ; Coen Ottenheijm [Pays-Bas] ; Yann Le Fur [France] ; Sébastien Banzet [France] ; Emilie Pecchi [France] ; Christophe Vilmen [France] ; Patrick J. Cozzone [France] ; Nathalie Koulmann [France] ; Edna C. Hardeman [Australie] ; David Bendahan [France] ; Julien Gondin [France]Source :
- PLoS ONE [ 1932-6203 ] ; 2014.
Abstract
Nemaline myopathy is the most common disease entity among non-dystrophic skeletal muscle congenital diseases. The first disease causing mutation (Met9Arg) was identified in the gene encoding α-tropomyosinslow gene (
Url:
DOI: 10.1371/journal.pone.0109066
PubMed: 25268244
PubMed Central: 4182639
Affiliations:
- Australie, France, Pays-Bas
- Hollande-Septentrionale, Nouvelle-Galles du Sud, Provence-Alpes-Côte d'Azur
- Amsterdam, Marseille, Sydney
- Université d'Aix-Marseille
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PMC:4182639Le document en format XML
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<author><name sortKey="Le Fur, Yann" sort="Le Fur, Yann" uniqKey="Le Fur Y" first="Yann" last="Le Fur">Yann Le Fur</name>
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<author><name sortKey="Banzet, Sebastien" sort="Banzet, Sebastien" uniqKey="Banzet S" first="Sébastien" last="Banzet">Sébastien Banzet</name>
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<author><name sortKey="Pecchi, Emilie" sort="Pecchi, Emilie" uniqKey="Pecchi E" first="Emilie" last="Pecchi">Emilie Pecchi</name>
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<author><name sortKey="Vilmen, Christophe" sort="Vilmen, Christophe" uniqKey="Vilmen C" first="Christophe" last="Vilmen">Christophe Vilmen</name>
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</nlm:aff>
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<author><name sortKey="Cozzone, Patrick J" sort="Cozzone, Patrick J" uniqKey="Cozzone P" first="Patrick J." last="Cozzone">Patrick J. Cozzone</name>
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</nlm:aff>
<country xml:lang="fr">France</country>
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<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
</author>
<author><name sortKey="Koulmann, Nathalie" sort="Koulmann, Nathalie" uniqKey="Koulmann N" first="Nathalie" last="Koulmann">Nathalie Koulmann</name>
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<author><name sortKey="Hardeman, Edna C" sort="Hardeman, Edna C" uniqKey="Hardeman E" first="Edna C." last="Hardeman">Edna C. Hardeman</name>
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</nlm:aff>
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<author><name sortKey="Bendahan, David" sort="Bendahan, David" uniqKey="Bendahan D" first="David" last="Bendahan">David Bendahan</name>
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</nlm:aff>
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<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
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<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
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<author><name sortKey="Gondin, Julien" sort="Gondin, Julien" uniqKey="Gondin J" first="Julien" last="Gondin">Julien Gondin</name>
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</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
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<settlement type="city">Marseille</settlement>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Alterations at the Cross-Bridge Level Are Associated with a Paradoxical Gain of Muscle Function <italic>In Vivo</italic>
in a Mouse Model of Nemaline Myopathy</title>
<author><name sortKey="Gineste, Charlotte" sort="Gineste, Charlotte" uniqKey="Gineste C" first="Charlotte" last="Gineste">Charlotte Gineste</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
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<settlement type="city">Marseille</settlement>
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<author><name sortKey="Ottenheijm, Coen" sort="Ottenheijm, Coen" uniqKey="Ottenheijm C" first="Coen" last="Ottenheijm">Coen Ottenheijm</name>
<affiliation wicri:level="3"><nlm:aff id="aff2"><addr-line>Department of Physiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands</addr-line>
</nlm:aff>
<country xml:lang="fr">Pays-Bas</country>
<wicri:regionArea>Department of Physiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam</wicri:regionArea>
<placeName><settlement type="city">Amsterdam</settlement>
<region nuts="2" type="province">Hollande-Septentrionale</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Le Fur, Yann" sort="Le Fur, Yann" uniqKey="Le Fur Y" first="Yann" last="Le Fur">Yann Le Fur</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
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<settlement type="city">Marseille</settlement>
<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
</author>
<author><name sortKey="Banzet, Sebastien" sort="Banzet, Sebastien" uniqKey="Banzet S" first="Sébastien" last="Banzet">Sébastien Banzet</name>
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</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Institut de Recherche Biomédicale des Armées, Physiologie des activités physiques, Brétigny sur Orge</wicri:regionArea>
<wicri:noRegion>Brétigny sur Orge</wicri:noRegion>
<wicri:noRegion>Brétigny sur Orge</wicri:noRegion>
</affiliation>
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<author><name sortKey="Pecchi, Emilie" sort="Pecchi, Emilie" uniqKey="Pecchi E" first="Emilie" last="Pecchi">Emilie Pecchi</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
<region type="old region">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
</author>
<author><name sortKey="Vilmen, Christophe" sort="Vilmen, Christophe" uniqKey="Vilmen C" first="Christophe" last="Vilmen">Christophe Vilmen</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
<region type="old region">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
</author>
<author><name sortKey="Cozzone, Patrick J" sort="Cozzone, Patrick J" uniqKey="Cozzone P" first="Patrick J." last="Cozzone">Patrick J. Cozzone</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
<region type="old region">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
</author>
<author><name sortKey="Koulmann, Nathalie" sort="Koulmann, Nathalie" uniqKey="Koulmann N" first="Nathalie" last="Koulmann">Nathalie Koulmann</name>
<affiliation wicri:level="1"><nlm:aff id="aff3"><addr-line>Institut de Recherche Biomédicale des Armées, Physiologie des activités physiques, Brétigny sur Orge, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Institut de Recherche Biomédicale des Armées, Physiologie des activités physiques, Brétigny sur Orge</wicri:regionArea>
<wicri:noRegion>Brétigny sur Orge</wicri:noRegion>
<wicri:noRegion>Brétigny sur Orge</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Hardeman, Edna C" sort="Hardeman, Edna C" uniqKey="Hardeman E" first="Edna C." last="Hardeman">Edna C. Hardeman</name>
<affiliation wicri:level="3"><nlm:aff id="aff4"><addr-line>School of Medical Sciences, University of New South Wales, Sydney, Australia</addr-line>
</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>School of Medical Sciences, University of New South Wales, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bendahan, David" sort="Bendahan, David" uniqKey="Bendahan D" first="David" last="Bendahan">David Bendahan</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
<region type="old region">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
</author>
<author><name sortKey="Gondin, Julien" sort="Gondin, Julien" uniqKey="Gondin J" first="Julien" last="Gondin">Julien Gondin</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille</wicri:regionArea>
<placeName><region type="region">Provence-Alpes-Côte d'Azur</region>
<region type="old region">Provence-Alpes-Côte d'Azur</region>
<settlement type="city">Marseille</settlement>
<settlement type="city">Marseille</settlement>
</placeName>
<orgName type="university">Université d'Aix-Marseille</orgName>
</affiliation>
</author>
</analytic>
<series><title level="j">PLoS ONE</title>
<idno type="eISSN">1932-6203</idno>
<imprint><date when="2014">2014</date>
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<front><div type="abstract" xml:lang="en"><p>Nemaline myopathy is the most common disease entity among non-dystrophic skeletal muscle congenital diseases. The first disease causing mutation (Met9Arg) was identified in the gene encoding α-tropomyosin<sub>slow</sub>
gene (<italic>TPM3</italic>
). Considering the conflicting findings of the previous studies on the transgenic (Tg) mice carrying the <italic>TPM3</italic>
<sup>Met9Arg</sup>
mutation, we investigated carefully the effect of the Met9Arg mutation in 8–9 month-old Tg(<italic>TPM3</italic>
)<sup>Met9Arg</sup>
mice on muscle function using a multiscale methodological approach including skinned muscle fibers analysis and <italic>in</italic>
<italic>vivo</italic>
investigations by magnetic resonance imaging and 31-phosphorus magnetic resonance spectroscopy. While <italic>in</italic>
<italic>vitro</italic>
maximal force production was reduced in Tg(<italic>TPM3</italic>
)<sup>Met9Arg</sup>
mice as compared to controls, <italic>in</italic>
<italic>vivo</italic>
measurements revealed an improved mechanical performance in the transgenic mice as compared to the former. The reduced <italic>in</italic>
<italic>vitro</italic>
muscle force might be related to alterations occuring at the cross-bridges level with muscle-specific underlying mechanisms. <italic>In vivo</italic>
muscle improvement was not associated with any changes in either muscle volume or energy metabolism. Our findings indicate that <italic>TPM3</italic>
(Met9Arg) mutation leads to a mild muscle weakness <italic>in</italic>
<italic>vitro</italic>
related to an alteration at the cross-bridges level and a paradoxical gain of muscle function <italic>in</italic>
<italic>vivo</italic>
. These results clearly point out that <italic>in</italic>
<italic>vitro</italic>
alterations are muscle-dependent and do not necessarily translate into similar changes <italic>in</italic>
<italic>vivo.</italic>
</p>
</div>
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<biblStruct><analytic><author><name sortKey="Periasamy, M" uniqKey="Periasamy M">M Periasamy</name>
</author>
<author><name sortKey="Bhupathy, P" uniqKey="Bhupathy P">P Bhupathy</name>
</author>
<author><name sortKey="Babu, Gj" uniqKey="Babu G">GJ Babu</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Tada, M" uniqKey="Tada M">M Tada</name>
</author>
<author><name sortKey="Inui, M" uniqKey="Inui M">M Inui</name>
</author>
</analytic>
</biblStruct>
</listBibl>
</div1>
</back>
</TEI>
<pmc article-type="research-article"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">PLoS One</journal-id>
<journal-id journal-id-type="iso-abbrev">PLoS ONE</journal-id>
<journal-id journal-id-type="publisher-id">plos</journal-id>
<journal-id journal-id-type="pmc">plosone</journal-id>
<journal-title-group><journal-title>PLoS ONE</journal-title>
</journal-title-group>
<issn pub-type="epub">1932-6203</issn>
<publisher><publisher-name>Public Library of Science</publisher-name>
<publisher-loc>San Francisco, USA</publisher-loc>
</publisher>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">25268244</article-id>
<article-id pub-id-type="pmc">4182639</article-id>
<article-id pub-id-type="publisher-id">PONE-D-14-01376</article-id>
<article-id pub-id-type="doi">10.1371/journal.pone.0109066</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject>
</subj-group>
<subj-group subj-group-type="Discipline-v2"><subject>Biology and Life Sciences</subject>
<subj-group><subject>Anatomy</subject>
<subj-group><subject>Musculoskeletal System</subject>
<subj-group><subject>Muscles</subject>
</subj-group>
</subj-group>
</subj-group>
</subj-group>
<subj-group subj-group-type="Discipline-v2"><subject>Medicine and Health Sciences</subject>
<subj-group><subject>Neurology</subject>
<subj-group><subject>Neuromuscular Diseases</subject>
</subj-group>
</subj-group>
</subj-group>
</article-categories>
<title-group><article-title>Alterations at the Cross-Bridge Level Are Associated with a Paradoxical Gain of Muscle Function <italic>In Vivo</italic>
in a Mouse Model of Nemaline Myopathy</article-title>
<alt-title alt-title-type="running-head"><italic>In Vivo</italic>
and <italic>In Vitro</italic>
Investigations of a NM Mouse Model</alt-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Gineste</surname>
<given-names>Charlotte</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Ottenheijm</surname>
<given-names>Coen</given-names>
</name>
<xref ref-type="aff" rid="aff2"><sup>2</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Le Fur</surname>
<given-names>Yann</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Banzet</surname>
<given-names>Sébastien</given-names>
</name>
<xref ref-type="aff" rid="aff3"><sup>3</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Pecchi</surname>
<given-names>Emilie</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Vilmen</surname>
<given-names>Christophe</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Cozzone</surname>
<given-names>Patrick J.</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Koulmann</surname>
<given-names>Nathalie</given-names>
</name>
<xref ref-type="aff" rid="aff3"><sup>3</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Hardeman</surname>
<given-names>Edna C.</given-names>
</name>
<xref ref-type="aff" rid="aff4"><sup>4</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Bendahan</surname>
<given-names>David</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Gondin</surname>
<given-names>Julien</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
<xref ref-type="corresp" rid="cor1"><sup>*</sup>
</xref>
</contrib>
</contrib-group>
<aff id="aff1"><label>1</label>
<addr-line>Aix-Marseille Université, Centre National de la Recherche Scientifique (CNRS), Centre de Résonance Magnétique Biologique et Médicale (CRMBM) Unité Mixte de Recherche (UMR), Marseille, France</addr-line>
</aff>
<aff id="aff2"><label>2</label>
<addr-line>Department of Physiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands</addr-line>
</aff>
<aff id="aff3"><label>3</label>
<addr-line>Institut de Recherche Biomédicale des Armées, Physiologie des activités physiques, Brétigny sur Orge, France</addr-line>
</aff>
<aff id="aff4"><label>4</label>
<addr-line>School of Medical Sciences, University of New South Wales, Sydney, Australia</addr-line>
</aff>
<contrib-group><contrib contrib-type="editor"><name><surname>Ervasti</surname>
<given-names>James M.</given-names>
</name>
<role>Editor</role>
<xref ref-type="aff" rid="edit1"></xref>
</contrib>
</contrib-group>
<aff id="edit1"><addr-line>University of Minnesota, United States of America</addr-line>
</aff>
<author-notes><corresp id="cor1">* E-mail: <email>julien.gondin@univ-amu.fr</email>
</corresp>
<fn fn-type="conflict"><p><bold>Competing Interests: </bold>
The authors have declared that no competing interests exist.</p>
</fn>
<fn fn-type="con"><p>Conceived and designed the experiments: CG JG. Performed the experiments: CG JG SB. Analyzed the data: CG JG SB. Contributed reagents/materials/analysis tools: YLF CV EP PJC ECH DB NK CO. Wrote the paper: CG JG.</p>
</fn>
</author-notes>
<pub-date pub-type="collection"><year>2014</year>
</pub-date>
<pub-date pub-type="epub"><day>30</day>
<month>9</month>
<year>2014</year>
</pub-date>
<volume>9</volume>
<issue>9</issue>
<elocation-id>e109066</elocation-id>
<history><date date-type="received"><day>10</day>
<month>1</month>
<year>2014</year>
</date>
<date date-type="accepted"><day>9</day>
<month>9</month>
<year>2014</year>
</date>
</history>
<permissions><copyright-year>2014</copyright-year>
<copyright-holder>Gineste et al</copyright-holder>
<license><license-p>This is an open-access article distributed under the terms of the <ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by/4.0/">Creative Commons Attribution License</ext-link>
, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.</license-p>
</license>
</permissions>
<abstract><p>Nemaline myopathy is the most common disease entity among non-dystrophic skeletal muscle congenital diseases. The first disease causing mutation (Met9Arg) was identified in the gene encoding α-tropomyosin<sub>slow</sub>
gene (<italic>TPM3</italic>
). Considering the conflicting findings of the previous studies on the transgenic (Tg) mice carrying the <italic>TPM3</italic>
<sup>Met9Arg</sup>
mutation, we investigated carefully the effect of the Met9Arg mutation in 8–9 month-old Tg(<italic>TPM3</italic>
)<sup>Met9Arg</sup>
mice on muscle function using a multiscale methodological approach including skinned muscle fibers analysis and <italic>in</italic>
<italic>vivo</italic>
investigations by magnetic resonance imaging and 31-phosphorus magnetic resonance spectroscopy. While <italic>in</italic>
<italic>vitro</italic>
maximal force production was reduced in Tg(<italic>TPM3</italic>
)<sup>Met9Arg</sup>
mice as compared to controls, <italic>in</italic>
<italic>vivo</italic>
measurements revealed an improved mechanical performance in the transgenic mice as compared to the former. The reduced <italic>in</italic>
<italic>vitro</italic>
muscle force might be related to alterations occuring at the cross-bridges level with muscle-specific underlying mechanisms. <italic>In vivo</italic>
muscle improvement was not associated with any changes in either muscle volume or energy metabolism. Our findings indicate that <italic>TPM3</italic>
(Met9Arg) mutation leads to a mild muscle weakness <italic>in</italic>
<italic>vitro</italic>
related to an alteration at the cross-bridges level and a paradoxical gain of muscle function <italic>in</italic>
<italic>vivo</italic>
. These results clearly point out that <italic>in</italic>
<italic>vitro</italic>
alterations are muscle-dependent and do not necessarily translate into similar changes <italic>in</italic>
<italic>vivo.</italic>
</p>
</abstract>
<funding-group><funding-statement>This work was supported by the ERA-NET E-Rare "European research projects on rare diseases" (grant NEMMYOP). Charlotte GINESTE was supported by a fellowship from the French Ministry of Higher Education and Research, the Association Française contre les Myopathies (AFM-TELETHON) and the Réseau franco-néerlandais. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.</funding-statement>
</funding-group>
<counts><page-count count="11"></page-count>
</counts>
</article-meta>
</front>
</pmc>
<affiliations><list><country><li>Australie</li>
<li>France</li>
<li>Pays-Bas</li>
</country>
<region><li>Hollande-Septentrionale</li>
<li>Nouvelle-Galles du Sud</li>
<li>Provence-Alpes-Côte d'Azur</li>
</region>
<settlement><li>Amsterdam</li>
<li>Marseille</li>
<li>Sydney</li>
</settlement>
<orgName><li>Université d'Aix-Marseille</li>
</orgName>
</list>
<tree><country name="France"><region name="Provence-Alpes-Côte d'Azur"><name sortKey="Gineste, Charlotte" sort="Gineste, Charlotte" uniqKey="Gineste C" first="Charlotte" last="Gineste">Charlotte Gineste</name>
</region>
<name sortKey="Banzet, Sebastien" sort="Banzet, Sebastien" uniqKey="Banzet S" first="Sébastien" last="Banzet">Sébastien Banzet</name>
<name sortKey="Bendahan, David" sort="Bendahan, David" uniqKey="Bendahan D" first="David" last="Bendahan">David Bendahan</name>
<name sortKey="Cozzone, Patrick J" sort="Cozzone, Patrick J" uniqKey="Cozzone P" first="Patrick J." last="Cozzone">Patrick J. Cozzone</name>
<name sortKey="Gondin, Julien" sort="Gondin, Julien" uniqKey="Gondin J" first="Julien" last="Gondin">Julien Gondin</name>
<name sortKey="Koulmann, Nathalie" sort="Koulmann, Nathalie" uniqKey="Koulmann N" first="Nathalie" last="Koulmann">Nathalie Koulmann</name>
<name sortKey="Le Fur, Yann" sort="Le Fur, Yann" uniqKey="Le Fur Y" first="Yann" last="Le Fur">Yann Le Fur</name>
<name sortKey="Pecchi, Emilie" sort="Pecchi, Emilie" uniqKey="Pecchi E" first="Emilie" last="Pecchi">Emilie Pecchi</name>
<name sortKey="Vilmen, Christophe" sort="Vilmen, Christophe" uniqKey="Vilmen C" first="Christophe" last="Vilmen">Christophe Vilmen</name>
</country>
<country name="Pays-Bas"><region name="Hollande-Septentrionale"><name sortKey="Ottenheijm, Coen" sort="Ottenheijm, Coen" uniqKey="Ottenheijm C" first="Coen" last="Ottenheijm">Coen Ottenheijm</name>
</region>
</country>
<country name="Australie"><region name="Nouvelle-Galles du Sud"><name sortKey="Hardeman, Edna C" sort="Hardeman, Edna C" uniqKey="Hardeman E" first="Edna C." last="Hardeman">Edna C. Hardeman</name>
</region>
</country>
</tree>
</affiliations>
</record>
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