Neuroglobin Gene Therapy Prevents Optic Atrophy and Preserves Durably Visual Function in Harlequin Mice
Identifieur interne : 001353 ( Ncbi/Merge ); précédent : 001352; suivant : 001354Neuroglobin Gene Therapy Prevents Optic Atrophy and Preserves Durably Visual Function in Harlequin Mice
Auteurs : Christophe Lechauve [France] ; Sébastien Augustin [France] ; Hélène Cwerman-Thibault [France] ; Élodie Reboussin [France] ; Delphine Roussel [France] ; René Lai-Kuen [France] ; Bruno Saubamea [France] ; José-Alain Sahel [France] ; Thomas Debeir [France] ; Marisol Corral-Debrinski [France]Source :
- Molecular Therapy [ 1525-0016 ] ; 2014.
Abstract
Neuroglobin (NGB) is considered as an endogenous neuroprotective molecule against stroke, since the protein alleviates the adverse effects of hypoxic and ischemic insults. We previously demonstrated the functional link between NGB and mitochondria since it is required for respiratory chain function. Thus, here, we evaluated the relevance of this effect in the
Url:
DOI: 10.1038/mt.2014.44
PubMed: 24622090
PubMed Central: 4048897
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strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2"><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff3"><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Augustin, Sebastien" sort="Augustin, Sebastien" uniqKey="Augustin S" first="Sébastien" last="Augustin">Sébastien Augustin</name><affiliation wicri:level="1"><nlm:aff id="aff1"><institution>INSERM, U968</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2"><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff3"><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Cwerman Thibault, Helene" sort="Cwerman Thibault, Helene" uniqKey="Cwerman Thibault H" first="Hélène" last="Cwerman-Thibault">Hélène Cwerman-Thibault</name><affiliation wicri:level="1"><nlm:aff id="aff1"><institution>INSERM, U968</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2"><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff3"><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Reboussin, Elodie" sort="Reboussin, Elodie" uniqKey="Reboussin E" first="Élodie" last="Reboussin">Élodie Reboussin</name><affiliation wicri:level="1"><nlm:aff id="aff1"><institution>INSERM, U968</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2"><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff3"><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Roussel, Delphine" sort="Roussel, Delphine" uniqKey="Roussel D" first="Delphine" last="Roussel">Delphine Roussel</name><affiliation wicri:level="1"><nlm:aff id="aff1"><institution>INSERM, U968</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2"><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff3"><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Lai Kuen, Rene" sort="Lai Kuen, Rene" uniqKey="Lai Kuen R" first="René" last="Lai-Kuen">René Lai-Kuen</name><affiliation wicri:level="1"><nlm:aff id="aff4"><institution>INSERM U705, CNRS UMR 8206, Université Paris Descartes, Sorbonne Paris Cité, Université Paris Diderot, Faculté de Pharmacie</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Saubamea, Bruno" sort="Saubamea, Bruno" uniqKey="Saubamea B" first="Bruno" last="Saubamea">Bruno Saubamea</name><affiliation wicri:level="1"><nlm:aff id="aff4"><institution>INSERM U705, CNRS UMR 8206, Université Paris Descartes, Sorbonne Paris Cité, Université Paris Diderot, Faculté de Pharmacie</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Sahel, Jose Alain" sort="Sahel, Jose Alain" uniqKey="Sahel J" first="José-Alain" last="Sahel">José-Alain Sahel</name><affiliation wicri:level="1"><nlm:aff id="aff1"><institution>INSERM, U968</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2"><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff3"><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Debeir, Thomas" sort="Debeir, Thomas" uniqKey="Debeir T" first="Thomas" last="Debeir">Thomas Debeir</name><affiliation wicri:level="1"><nlm:aff id="aff5"><institution>Sanofi Fovea – Ophthalmology</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author><author><name sortKey="Corral Debrinski, Marisol" sort="Corral Debrinski, Marisol" uniqKey="Corral Debrinski M" first="Marisol" last="Corral-Debrinski">Marisol Corral-Debrinski</name><affiliation wicri:level="1"><nlm:aff id="aff1"><institution>INSERM, U968</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2"><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:aff id="aff3"><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></nlm:aff><country xml:lang="fr">France</country><wicri:regionArea># see nlm:aff country strict</wicri:regionArea></affiliation></author></analytic><series><title level="j">Molecular Therapy</title><idno type="ISSN">1525-0016</idno><idno type="eISSN">1525-0024</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>Neuroglobin (NGB) is considered as an endogenous neuroprotective molecule against stroke, since the protein alleviates the adverse effects of hypoxic and ischemic insults. We previously demonstrated the functional link between NGB and mitochondria since it is required for respiratory chain function. Thus, here, we evaluated the relevance of this effect in the <italic>Harlequin</italic> (<italic>Hq</italic>) mouse strain, which exhibits retinal ganglion cell (RGC) loss and optic atrophy due to a respiratory chain complex I (CI) defect. A twofold decrease of NGB amounts was observed in <italic>Hq</italic> retinas. We constructed a recombinant adeno-associated virus which combines to the mouse NGB open reading frame, its 5′ and 3′UTR, for guarantying mRNA stability and translation capacity. The vector was administrated intravitreally to <italic>Hq</italic> mice and NGB expression was stable for up to 7 months without negative effect on retinal architecture or function. On the contrary, RGCs and their axons were substantially preserved from degeneration; consequently, CI activity in optic nerves was protected conferring improvements in vision. Hence, we established that NGB prevents respiratory chain impairment, therefore, protecting visual function otherwise compromised by mitochondrial energetic failure.</p></div></front></TEI><pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Mol Ther</journal-id><journal-id journal-id-type="iso-abbrev">Mol. Ther</journal-id><journal-title-group><journal-title>Molecular Therapy</journal-title></journal-title-group><issn pub-type="ppub">1525-0016</issn><issn pub-type="epub">1525-0024</issn><publisher><publisher-name>Nature Publishing Group</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">24622090</article-id><article-id pub-id-type="pmc">4048897</article-id><article-id pub-id-type="pii">mt201444</article-id><article-id pub-id-type="doi">10.1038/mt.2014.44</article-id><article-categories><subj-group subj-group-type="heading"><subject>Original Article</subject></subj-group></article-categories><title-group><article-title>Neuroglobin Gene Therapy Prevents Optic Atrophy and Preserves Durably Visual Function in <italic>Harlequin</italic> Mice</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Lechauve</surname><given-names>Christophe</given-names></name><xref ref-type="aff" rid="aff1">1</xref><xref ref-type="aff" rid="aff2">2</xref><xref ref-type="aff" rid="aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Augustin</surname><given-names>Sébastien</given-names></name><xref ref-type="aff" rid="aff1">1</xref><xref ref-type="aff" rid="aff2">2</xref><xref ref-type="aff" rid="aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Cwerman-Thibault</surname><given-names>Hélène</given-names></name><xref ref-type="aff" rid="aff1">1</xref><xref ref-type="aff" rid="aff2">2</xref><xref ref-type="aff" rid="aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Reboussin</surname><given-names>Élodie</given-names></name><xref ref-type="aff" rid="aff1">1</xref><xref ref-type="aff" rid="aff2">2</xref><xref ref-type="aff" rid="aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Roussel</surname><given-names>Delphine</given-names></name><xref ref-type="aff" rid="aff1">1</xref><xref ref-type="aff" rid="aff2">2</xref><xref ref-type="aff" rid="aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Lai-Kuen</surname><given-names>René</given-names></name><xref ref-type="aff" rid="aff4">4</xref></contrib><contrib contrib-type="author"><name><surname>Saubamea</surname><given-names>Bruno</given-names></name><xref ref-type="aff" rid="aff4">4</xref></contrib><contrib contrib-type="author"><name><surname>Sahel</surname><given-names>José-Alain</given-names></name><xref ref-type="aff" rid="aff1">1</xref><xref ref-type="aff" rid="aff2">2</xref><xref ref-type="aff" rid="aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Debeir</surname><given-names>Thomas</given-names></name><xref ref-type="aff" rid="aff5">5</xref></contrib><contrib contrib-type="author"><name><surname>Corral-Debrinski</surname><given-names>Marisol</given-names></name><xref ref-type="aff" rid="aff1">1</xref><xref ref-type="aff" rid="aff2">2</xref><xref ref-type="aff" rid="aff3">3</xref><xref ref-type="corresp" rid="caf1">*</xref></contrib><aff id="aff1"><label>1</label><institution>INSERM, U968</institution>, Paris,<country>France</country></aff><aff id="aff2"><label>2</label><institution>Sorbonne Universités, UPMC Univ Paris, Institut de la Vision</institution>, Paris,<country>France</country></aff><aff id="aff3"><label>3</label><institution>Centre national de la recherche scientifique</institution>, Paris,<country>France</country></aff><aff id="aff4"><label>4</label><institution>INSERM U705, CNRS UMR 8206, Université Paris Descartes, Sorbonne Paris Cité, Université Paris Diderot, Faculté de Pharmacie</institution>, Paris,<country>France</country></aff><aff id="aff5"><label>5</label><institution>Sanofi Fovea – Ophthalmology</institution>, Paris,<country>France</country></aff></contrib-group><author-notes><corresp id="caf1"><label>*</label>INSERM, U968, Paris, F-75012, France. E-mail: <email>marisol.corral@inserm.fr</email></corresp><fn fn-type="present-address" id="aunote"><label></label><p>H.C-T. and E.R. contributed equally to this work.</p></fn></author-notes><pub-date pub-type="ppub"><month>06</month><year>2014</year></pub-date><pub-date pub-type="epreprint"><day>13</day><month>03</month><year>2014</year></pub-date><pub-date pub-type="epub"><day>08</day><month>04</month><year>2014</year></pub-date><volume>22</volume><issue>6</issue><fpage>1096</fpage><lpage>1109</lpage><history><date date-type="received"><day>23</day><month>01</month><year>2014</year></date><date date-type="accepted"><day>06</day><month>03</month><year>2014</year></date></history><permissions><copyright-statement>Copyright © 2014 The American Society of Gene & Cell Therapy</copyright-statement><copyright-year>2014</copyright-year><copyright-holder>The American Society of Gene & Cell Therapy</copyright-holder></permissions><abstract><p>Neuroglobin (NGB) is considered as an endogenous neuroprotective molecule against stroke, since the protein alleviates the adverse effects of hypoxic and ischemic insults. We previously demonstrated the functional link between NGB and mitochondria since it is required for respiratory chain function. Thus, here, we evaluated the relevance of this effect in the <italic>Harlequin</italic> (<italic>Hq</italic>) mouse strain, which exhibits retinal ganglion cell (RGC) loss and optic atrophy due to a respiratory chain complex I (CI) defect. A twofold decrease of NGB amounts was observed in <italic>Hq</italic> retinas. We constructed a recombinant adeno-associated virus which combines to the mouse NGB open reading frame, its 5′ and 3′UTR, for guarantying mRNA stability and translation capacity. The vector was administrated intravitreally to <italic>Hq</italic> mice and NGB expression was stable for up to 7 months without negative effect on retinal architecture or function. On the contrary, RGCs and their axons were substantially preserved from degeneration; consequently, CI activity in optic nerves was protected conferring improvements in vision. Hence, we established that NGB prevents respiratory chain impairment, therefore, protecting visual function otherwise compromised by mitochondrial energetic failure.</p></abstract></article-meta></front></pmc><affiliations><list><country><li>France</li></country></list><tree><country name="France"><noRegion><name sortKey="Lechauve, Christophe" sort="Lechauve, Christophe" uniqKey="Lechauve C" first="Christophe" last="Lechauve">Christophe Lechauve</name></noRegion><name sortKey="Augustin, Sebastien" sort="Augustin, Sebastien" uniqKey="Augustin S" first="Sébastien" last="Augustin">Sébastien Augustin</name><name sortKey="Augustin, Sebastien" sort="Augustin, Sebastien" uniqKey="Augustin S" first="Sébastien" last="Augustin">Sébastien Augustin</name><name sortKey="Augustin, Sebastien" sort="Augustin, Sebastien" uniqKey="Augustin S" first="Sébastien" last="Augustin">Sébastien Augustin</name><name sortKey="Corral Debrinski, Marisol" sort="Corral Debrinski, Marisol" uniqKey="Corral Debrinski M" first="Marisol" last="Corral-Debrinski">Marisol Corral-Debrinski</name><name sortKey="Corral Debrinski, Marisol" sort="Corral Debrinski, Marisol" uniqKey="Corral Debrinski M" first="Marisol" last="Corral-Debrinski">Marisol Corral-Debrinski</name><name sortKey="Corral Debrinski, Marisol" sort="Corral Debrinski, Marisol" uniqKey="Corral Debrinski M" first="Marisol" last="Corral-Debrinski">Marisol Corral-Debrinski</name><name sortKey="Cwerman Thibault, Helene" sort="Cwerman Thibault, Helene" uniqKey="Cwerman Thibault H" first="Hélène" last="Cwerman-Thibault">Hélène Cwerman-Thibault</name><name sortKey="Cwerman Thibault, Helene" sort="Cwerman Thibault, Helene" uniqKey="Cwerman Thibault H" first="Hélène" last="Cwerman-Thibault">Hélène Cwerman-Thibault</name><name sortKey="Cwerman Thibault, Helene" sort="Cwerman Thibault, Helene" uniqKey="Cwerman Thibault H" first="Hélène" last="Cwerman-Thibault">Hélène Cwerman-Thibault</name><name sortKey="Debeir, Thomas" sort="Debeir, Thomas" uniqKey="Debeir T" first="Thomas" last="Debeir">Thomas Debeir</name><name sortKey="Lai Kuen, Rene" sort="Lai Kuen, Rene" uniqKey="Lai Kuen R" first="René" last="Lai-Kuen">René Lai-Kuen</name><name sortKey="Lechauve, Christophe" sort="Lechauve, Christophe" uniqKey="Lechauve C" first="Christophe" last="Lechauve">Christophe Lechauve</name><name sortKey="Lechauve, Christophe" sort="Lechauve, Christophe" uniqKey="Lechauve C" first="Christophe" last="Lechauve">Christophe Lechauve</name><name sortKey="Reboussin, Elodie" sort="Reboussin, Elodie" uniqKey="Reboussin E" first="Élodie" last="Reboussin">Élodie Reboussin</name><name sortKey="Reboussin, Elodie" sort="Reboussin, Elodie" uniqKey="Reboussin E" first="Élodie" last="Reboussin">Élodie Reboussin</name><name sortKey="Reboussin, Elodie" sort="Reboussin, Elodie" uniqKey="Reboussin E" first="Élodie" last="Reboussin">Élodie Reboussin</name><name sortKey="Roussel, Delphine" sort="Roussel, Delphine" uniqKey="Roussel D" first="Delphine" last="Roussel">Delphine Roussel</name><name sortKey="Roussel, Delphine" sort="Roussel, Delphine" uniqKey="Roussel D" first="Delphine" last="Roussel">Delphine Roussel</name><name sortKey="Roussel, Delphine" sort="Roussel, Delphine" uniqKey="Roussel D" first="Delphine" last="Roussel">Delphine Roussel</name><name sortKey="Sahel, Jose Alain" sort="Sahel, Jose Alain" uniqKey="Sahel J" first="José-Alain" last="Sahel">José-Alain Sahel</name><name sortKey="Sahel, Jose Alain" sort="Sahel, Jose Alain" uniqKey="Sahel J" first="José-Alain" last="Sahel">José-Alain Sahel</name><name sortKey="Sahel, Jose Alain" sort="Sahel, Jose Alain" uniqKey="Sahel J" first="José-Alain" last="Sahel">José-Alain Sahel</name><name sortKey="Saubamea, Bruno" sort="Saubamea, Bruno" uniqKey="Saubamea B" first="Bruno" last="Saubamea">Bruno Saubamea</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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