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 : 001E55 ( Ncbi/Merge ); précédent : 001E54; suivant : 001E56Alterations 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.
Descripteurs français
- KwdFr :
- Adénosine triphosphate (métabolisme), Animaux, Faiblesse musculaire (anatomopathologie), Faiblesse musculaire (métabolisme), Faiblesse musculaire (physiopathologie), Imagerie par résonance magnétique, Modèles animaux de maladie humaine, Muscles squelettiques (anatomopathologie), Muscles squelettiques (métabolisme), Muscles squelettiques (physiopathologie), Mutation, Myopathies némaline (anatomopathologie), Myopathies némaline (génétique), Myopathies némaline (métabolisme), Myopathies némaline (physiopathologie), Métabolisme énergétique, Phénomènes biomécaniques, Souris, Souris transgéniques, Tropomyosine (génétique), Troponine I (métabolisme).
- MESH :
- anatomopathologie : Faiblesse musculaire, Muscles squelettiques, Myopathies némaline.
- génétique : Myopathies némaline, Tropomyosine.
- métabolisme : Adénosine triphosphate, Faiblesse musculaire, Muscles squelettiques, Myopathies némaline, Troponine I.
- physiopathologie : Faiblesse musculaire, Muscles squelettiques, Myopathies némaline.
- Animaux, Imagerie par résonance magnétique, Modèles animaux de maladie humaine, Mutation, Métabolisme énergétique, Phénomènes biomécaniques, Souris, Souris transgéniques.
English descriptors
- KwdEn :
- Adenosine Triphosphate (metabolism), Animals, Biomechanical Phenomena, Disease Models, Animal, Energy Metabolism, Magnetic Resonance Imaging, Mice, Mice, Transgenic, Muscle Weakness (metabolism), Muscle Weakness (pathology), Muscle Weakness (physiopathology), Muscle, Skeletal (metabolism), Muscle, Skeletal (pathology), Muscle, Skeletal (physiopathology), Mutation, Myopathies, Nemaline (genetics), Myopathies, Nemaline (metabolism), Myopathies, Nemaline (pathology), Myopathies, Nemaline (physiopathology), Tropomyosin (genetics), Troponin I (metabolism).
- MESH :
- chemical , genetics : Tropomyosin.
- chemical , metabolism : Adenosine Triphosphate, Troponin I.
- genetics : Myopathies, Nemaline.
- metabolism : Muscle Weakness, Muscle, Skeletal, Myopathies, Nemaline.
- pathology : Muscle Weakness, Muscle, Skeletal, Myopathies, Nemaline.
- physiopathology : Muscle Weakness, Muscle, Skeletal, Myopathies, Nemaline.
- Animals, Biomechanical Phenomena, Disease Models, Animal, Energy Metabolism, Magnetic Resonance Imaging, Mice, Mice, Transgenic, Mutation.
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
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PMC:4182639Le document en format XML
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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>
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</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>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adenosine Triphosphate (metabolism)</term>
<term>Animals</term>
<term>Biomechanical Phenomena</term>
<term>Disease Models, Animal</term>
<term>Energy Metabolism</term>
<term>Magnetic Resonance Imaging</term>
<term>Mice</term>
<term>Mice, Transgenic</term>
<term>Muscle Weakness (metabolism)</term>
<term>Muscle Weakness (pathology)</term>
<term>Muscle Weakness (physiopathology)</term>
<term>Muscle, Skeletal (metabolism)</term>
<term>Muscle, Skeletal (pathology)</term>
<term>Muscle, Skeletal (physiopathology)</term>
<term>Mutation</term>
<term>Myopathies, Nemaline (genetics)</term>
<term>Myopathies, Nemaline (metabolism)</term>
<term>Myopathies, Nemaline (pathology)</term>
<term>Myopathies, Nemaline (physiopathology)</term>
<term>Tropomyosin (genetics)</term>
<term>Troponin I (metabolism)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adénosine triphosphate (métabolisme)</term>
<term>Animaux</term>
<term>Faiblesse musculaire (anatomopathologie)</term>
<term>Faiblesse musculaire (métabolisme)</term>
<term>Faiblesse musculaire (physiopathologie)</term>
<term>Imagerie par résonance magnétique</term>
<term>Modèles animaux de maladie humaine</term>
<term>Muscles squelettiques (anatomopathologie)</term>
<term>Muscles squelettiques (métabolisme)</term>
<term>Muscles squelettiques (physiopathologie)</term>
<term>Mutation</term>
<term>Myopathies némaline (anatomopathologie)</term>
<term>Myopathies némaline (génétique)</term>
<term>Myopathies némaline (métabolisme)</term>
<term>Myopathies némaline (physiopathologie)</term>
<term>Métabolisme énergétique</term>
<term>Phénomènes biomécaniques</term>
<term>Souris</term>
<term>Souris transgéniques</term>
<term>Tropomyosine (génétique)</term>
<term>Troponine I (métabolisme)</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Tropomyosin</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Adenosine Triphosphate</term>
<term>Troponin I</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Faiblesse musculaire</term>
<term>Muscles squelettiques</term>
<term>Myopathies némaline</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Myopathies némaline</term>
<term>Tropomyosine</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Muscle Weakness</term>
<term>Muscle, Skeletal</term>
<term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Adénosine triphosphate</term>
<term>Faiblesse musculaire</term>
<term>Muscles squelettiques</term>
<term>Myopathies némaline</term>
<term>Troponine I</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Muscle Weakness</term>
<term>Muscle, Skeletal</term>
<term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathologie" xml:lang="fr"><term>Faiblesse musculaire</term>
<term>Muscles squelettiques</term>
<term>Myopathies némaline</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Muscle Weakness</term>
<term>Muscle, Skeletal</term>
<term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Animals</term>
<term>Biomechanical Phenomena</term>
<term>Disease Models, Animal</term>
<term>Energy Metabolism</term>
<term>Magnetic Resonance Imaging</term>
<term>Mice</term>
<term>Mice, Transgenic</term>
<term>Mutation</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Animaux</term>
<term>Imagerie par résonance magnétique</term>
<term>Modèles animaux de maladie humaine</term>
<term>Mutation</term>
<term>Métabolisme énergétique</term>
<term>Phénomènes biomécaniques</term>
<term>Souris</term>
<term>Souris transgéniques</term>
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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>
</front>
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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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</nlm:aff>
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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>
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</nlm:aff>
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</author>
<author><name sortKey="Ottenheijm, Coen" sort="Ottenheijm, Coen" uniqKey="Ottenheijm C" first="Coen" last="Ottenheijm">Coen Ottenheijm</name>
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</nlm:aff>
<country xml:lang="fr">Pays-Bas</country>
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<placeName><settlement type="city">Amsterdam</settlement>
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</author>
<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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</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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<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>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>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<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>
</front>
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<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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<settlement type="city">Marseille</settlement>
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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>
<affiliation wicri:level="4"><nlm:affiliation>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.</nlm:affiliation>
<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>
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</author>
<author><name sortKey="Koulmann, Nathalie" sort="Koulmann, Nathalie" uniqKey="Koulmann N" first="Nathalie" last="Koulmann">Nathalie Koulmann</name>
<affiliation wicri:level="1"><nlm:affiliation>Institut de Recherche Biomédicale des Armées, Physiologie des activités physiques, Brétigny sur Orge, France.</nlm:affiliation>
<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:affiliation>School of Medical Sciences, University of New South Wales, Sydney, Australia.</nlm:affiliation>
<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:affiliation>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.</nlm:affiliation>
<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>
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</author>
<author><name sortKey="Gondin, Julien" sort="Gondin, Julien" uniqKey="Gondin J" first="Julien" last="Gondin">Julien Gondin</name>
<affiliation wicri:level="4"><nlm:affiliation>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.</nlm:affiliation>
<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>
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</analytic>
<series><title level="j">PloS one</title>
<idno type="eISSN">1932-6203</idno>
<imprint><date when="2014" type="published">2014</date>
</imprint>
</series>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adenosine Triphosphate (metabolism)</term>
<term>Animals</term>
<term>Biomechanical Phenomena</term>
<term>Disease Models, Animal</term>
<term>Energy Metabolism</term>
<term>Magnetic Resonance Imaging</term>
<term>Mice</term>
<term>Mice, Transgenic</term>
<term>Muscle Weakness (metabolism)</term>
<term>Muscle Weakness (pathology)</term>
<term>Muscle Weakness (physiopathology)</term>
<term>Muscle, Skeletal (metabolism)</term>
<term>Muscle, Skeletal (pathology)</term>
<term>Muscle, Skeletal (physiopathology)</term>
<term>Mutation</term>
<term>Myopathies, Nemaline (genetics)</term>
<term>Myopathies, Nemaline (metabolism)</term>
<term>Myopathies, Nemaline (pathology)</term>
<term>Myopathies, Nemaline (physiopathology)</term>
<term>Tropomyosin (genetics)</term>
<term>Troponin I (metabolism)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adénosine triphosphate (métabolisme)</term>
<term>Animaux</term>
<term>Faiblesse musculaire (anatomopathologie)</term>
<term>Faiblesse musculaire (métabolisme)</term>
<term>Faiblesse musculaire (physiopathologie)</term>
<term>Imagerie par résonance magnétique</term>
<term>Modèles animaux de maladie humaine</term>
<term>Muscles squelettiques (anatomopathologie)</term>
<term>Muscles squelettiques (métabolisme)</term>
<term>Muscles squelettiques (physiopathologie)</term>
<term>Mutation</term>
<term>Myopathies némaline (anatomopathologie)</term>
<term>Myopathies némaline (génétique)</term>
<term>Myopathies némaline (métabolisme)</term>
<term>Myopathies némaline (physiopathologie)</term>
<term>Métabolisme énergétique</term>
<term>Phénomènes biomécaniques</term>
<term>Souris</term>
<term>Souris transgéniques</term>
<term>Tropomyosine (génétique)</term>
<term>Troponine I (métabolisme)</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Tropomyosin</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Adenosine Triphosphate</term>
<term>Troponin I</term>
</keywords>
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<term>Muscles squelettiques</term>
<term>Myopathies némaline</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Myopathies némaline</term>
<term>Tropomyosine</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Muscle Weakness</term>
<term>Muscle, Skeletal</term>
<term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Adénosine triphosphate</term>
<term>Faiblesse musculaire</term>
<term>Muscles squelettiques</term>
<term>Myopathies némaline</term>
<term>Troponine I</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Muscle Weakness</term>
<term>Muscle, Skeletal</term>
<term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathologie" xml:lang="fr"><term>Faiblesse musculaire</term>
<term>Muscles squelettiques</term>
<term>Myopathies némaline</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Muscle Weakness</term>
<term>Muscle, Skeletal</term>
<term>Myopathies, Nemaline</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Animals</term>
<term>Biomechanical Phenomena</term>
<term>Disease Models, Animal</term>
<term>Energy Metabolism</term>
<term>Magnetic Resonance Imaging</term>
<term>Mice</term>
<term>Mice, Transgenic</term>
<term>Mutation</term>
</keywords>
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<term>Imagerie par résonance magnétique</term>
<term>Modèles animaux de maladie humaine</term>
<term>Mutation</term>
<term>Métabolisme énergétique</term>
<term>Phénomènes biomécaniques</term>
<term>Souris</term>
<term>Souris transgéniques</term>
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<front><div type="abstract" xml:lang="en">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 slow gene (TPM3). Considering the conflicting findings of the previous studies on the transgenic (Tg) mice carrying the TPM3Met9Arg mutation, we investigated carefully the effect of the Met9Arg mutation in 8-9 month-old Tg(TPM3)Met9Arg mice on muscle function using a multiscale methodological approach including skinned muscle fibers analysis and in vivo investigations by magnetic resonance imaging and 31-phosphorus magnetic resonance spectroscopy. While in vitro maximal force production was reduced in Tg(TPM3)Met9Arg mice as compared to controls, in vivo measurements revealed an improved mechanical performance in the transgenic mice as compared to the former. The reduced in vitro muscle force might be related to alterations occurring at the cross-bridges level with muscle-specific underlying mechanisms. In vivo muscle improvement was not associated with any changes in either muscle volume or energy metabolism. Our findings indicate that TPM3(Met9Arg) mutation leads to a mild muscle weakness in vitro related to an alteration at the cross-bridges level and a paradoxical gain of muscle function in vivo. These results clearly point out that in vitro alterations are muscle-dependent and do not necessarily translate into similar changes in vivo.</div>
</front>
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