Pathologic and Phenotypic Alterations in a Mouse Expressing a Connexin47 Missense Mutation That Causes Pelizaeus-Merzbacher–Like Disease in Humans
Identifieur interne : 004518 ( Ncbi/Merge ); précédent : 004517; suivant : 004519Pathologic and Phenotypic Alterations in a Mouse Expressing a Connexin47 Missense Mutation That Causes Pelizaeus-Merzbacher–Like Disease in Humans
Auteurs : Oliver Tress [Allemagne] ; Marta Maglione [Allemagne] ; Armin Zlomuzica [Allemagne] ; Dennis May [Allemagne] ; Nikolai Dicke [Allemagne] ; Joachim Degen [Allemagne] ; Ekrem Dere [France] ; Helmut Kettenmann [Allemagne] ; Dieter Hartmann [Allemagne] ; Klaus Willecke [Allemagne]Source :
- PLoS Genetics [ 1553-7390 ] ; 2011.
Abstract
Gap junction channels are intercellular conduits that allow diffusional exchange of ions, second messengers, and metabolites. Human oligodendrocytes express the gap junction protein connexin47 (Cx47), which is encoded by the
Url:
DOI: 10.1371/journal.pgen.1002146
PubMed: 21750683
PubMed Central: 3131295
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<author><name sortKey="Willecke, Klaus" sort="Willecke, Klaus" uniqKey="Willecke K" first="Klaus" last="Willecke">Klaus Willecke</name>
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</nlm:aff>
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<front><div type="abstract" xml:lang="en"><p>Gap junction channels are intercellular conduits that allow diffusional exchange of ions, second messengers, and metabolites. Human oligodendrocytes express the gap junction protein connexin47 (Cx47), which is encoded by the <italic>GJC2</italic>
gene. The autosomal recessive mutation hCx47M283T causes Pelizaeus-Merzbacher–like disease 1 (PMLD1), a progressive leukodystrophy characterized by hypomyelination, retarded motor development, nystagmus, and spasticity. We introduced the human missense mutation into the orthologous position of the mouse <italic>Gjc2</italic>
gene and inserted the <italic>mCx47M282T</italic>
coding sequence into the mouse genome via homologous recombination in embryonic stem cells. Three-week-old homozygous <italic>Cx47M282T</italic>
mice displayed impaired rotarod performance but unchanged open-field behavior. 10-15-day-old homozygous <italic>Cx47M282T</italic>
and Cx47 null mice revealed a more than 80% reduction in the number of cells participating in glial networks after biocytin injections into oligodendrocytes in sections of corpus callosum. Homozygous expression of <italic>mCx47M282T</italic>
resulted in reduced MBP expression and astrogliosis in the cerebellum of ten-day-old mice which could also be detected in Cx47 null mice of the same age. Three-month-old homozygous <italic>Cx47M282T</italic>
mice exhibited neither altered open-field behavior nor impaired rotarod performance anymore. Adult <italic>mCx47M282T</italic>
expressing mice did not show substantial myelin alterations, but homozygous <italic>Cx47M282T</italic>
mice, additionally deprived of connexin32, which is also expressed in oligodendrocytes, died within six weeks after birth and displayed severe myelin defects accompanied by astrogliosis and activated microglia. These results strongly suggest that PMLD1 is caused by the loss of Cx47 channel function that results in impaired panglial coupling in white matter tissue.</p>
</div>
</front>
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<pmc article-type="research-article"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">PLoS Genet</journal-id>
<journal-id journal-id-type="iso-abbrev">PLoS Genet</journal-id>
<journal-id journal-id-type="publisher-id">plos</journal-id>
<journal-id journal-id-type="pmc">plosgen</journal-id>
<journal-title-group><journal-title>PLoS Genetics</journal-title>
</journal-title-group>
<issn pub-type="ppub">1553-7390</issn>
<issn pub-type="epub">1553-7404</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">21750683</article-id>
<article-id pub-id-type="pmc">3131295</article-id>
<article-id pub-id-type="publisher-id">PGENETICS-D-10-00404</article-id>
<article-id pub-id-type="doi">10.1371/journal.pgen.1002146</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</subject>
<subj-group><subject>Anatomy and Physiology</subject>
</subj-group>
<subj-group><subject>Genetics</subject>
</subj-group>
<subj-group><subject>Neuroscience</subject>
</subj-group>
</subj-group>
<subj-group subj-group-type="Discipline-v2"><subject>Medicine</subject>
<subj-group><subject>Clinical Genetics</subject>
</subj-group>
<subj-group><subject>Neurology</subject>
</subj-group>
<subj-group><subject>Pediatrics</subject>
</subj-group>
</subj-group>
</article-categories>
<title-group><article-title>Pathologic and Phenotypic Alterations in a Mouse Expressing a Connexin47 Missense Mutation That Causes Pelizaeus-Merzbacher–Like Disease in Humans</article-title>
<alt-title alt-title-type="running-head">The Connexin47 M282T Mutant Mouse</alt-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Tress</surname>
<given-names>Oliver</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Maglione</surname>
<given-names>Marta</given-names>
</name>
<xref ref-type="aff" rid="aff2"><sup>2</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Zlomuzica</surname>
<given-names>Armin</given-names>
</name>
<xref ref-type="aff" rid="aff3"><sup>3</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>May</surname>
<given-names>Dennis</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Dicke</surname>
<given-names>Nikolai</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Degen</surname>
<given-names>Joachim</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Dere</surname>
<given-names>Ekrem</given-names>
</name>
<xref ref-type="aff" rid="aff4"><sup>4</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Kettenmann</surname>
<given-names>Helmut</given-names>
</name>
<xref ref-type="aff" rid="aff2"><sup>2</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Hartmann</surname>
<given-names>Dieter</given-names>
</name>
<xref ref-type="aff" rid="aff5"><sup>5</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Willecke</surname>
<given-names>Klaus</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>Institute of Genetics, Division of Molecular Genetics, University of Bonn, Bonn, Germany</addr-line>
</aff>
<aff id="aff2"><label>2</label>
<addr-line>Cellular Neurosciences, Max-Delbrück Center for Molecular Medicine, Berlin, Germany</addr-line>
</aff>
<aff id="aff3"><label>3</label>
<addr-line>Center for the Study and Treatment of Mental Health, Ruhr-Universität Bochum, Bochum, Germany</addr-line>
</aff>
<aff id="aff4"><label>4</label>
<addr-line>Université Pierre et Marie Curie (Paris VI), UMR 7102, Neurobiologie des Processus Adaptatifs, Paris, France</addr-line>
</aff>
<aff id="aff5"><label>5</label>
<addr-line>Department of Anatomy, Division of Neuroanatomy, University of Bonn, Bonn, Germany</addr-line>
</aff>
<contrib-group><contrib contrib-type="editor"><name><surname>Cox</surname>
<given-names>Gregory A.</given-names>
</name>
<role>Editor</role>
<xref ref-type="aff" rid="edit1"></xref>
</contrib>
</contrib-group>
<aff id="edit1">The Jackson Laboratory, United States of America</aff>
<author-notes><corresp id="cor1">* E-mail: <email>k.willecke@uni-bonn.de</email>
</corresp>
<fn fn-type="con"><p>Conceived and designed the experiments: OT MM AZ ED DH HK KW. Performed the experiments: OT MM AZ DM DH JD. Analyzed the data: OT MM AZ ND DH. Contributed reagents/materials/analysis tools: ED DH HK KW. Wrote the paper: OT MM ED DH KW. Concept, initiation, and organization of the project: KW. </p>
</fn>
</author-notes>
<pub-date pub-type="collection"><month>7</month>
<year>2011</year>
</pub-date>
<pub-date pub-type="epub"><day>7</day>
<month>7</month>
<year>2011</year>
</pub-date>
<volume>7</volume>
<issue>7</issue>
<elocation-id>e1002146</elocation-id>
<history><date date-type="received"><day>24</day>
<month>11</month>
<year>2010</year>
</date>
<date date-type="accepted"><day>1</day>
<month>5</month>
<year>2011</year>
</date>
</history>
<permissions><copyright-statement>Tress et al.</copyright-statement>
<copyright-year>2011</copyright-year>
<license xlink:href="http://creativecommons.org/licenses/by/4.0/"><license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.</license-p>
</license>
</permissions>
<abstract><p>Gap junction channels are intercellular conduits that allow diffusional exchange of ions, second messengers, and metabolites. Human oligodendrocytes express the gap junction protein connexin47 (Cx47), which is encoded by the <italic>GJC2</italic>
gene. The autosomal recessive mutation hCx47M283T causes Pelizaeus-Merzbacher–like disease 1 (PMLD1), a progressive leukodystrophy characterized by hypomyelination, retarded motor development, nystagmus, and spasticity. We introduced the human missense mutation into the orthologous position of the mouse <italic>Gjc2</italic>
gene and inserted the <italic>mCx47M282T</italic>
coding sequence into the mouse genome via homologous recombination in embryonic stem cells. Three-week-old homozygous <italic>Cx47M282T</italic>
mice displayed impaired rotarod performance but unchanged open-field behavior. 10-15-day-old homozygous <italic>Cx47M282T</italic>
and Cx47 null mice revealed a more than 80% reduction in the number of cells participating in glial networks after biocytin injections into oligodendrocytes in sections of corpus callosum. Homozygous expression of <italic>mCx47M282T</italic>
resulted in reduced MBP expression and astrogliosis in the cerebellum of ten-day-old mice which could also be detected in Cx47 null mice of the same age. Three-month-old homozygous <italic>Cx47M282T</italic>
mice exhibited neither altered open-field behavior nor impaired rotarod performance anymore. Adult <italic>mCx47M282T</italic>
expressing mice did not show substantial myelin alterations, but homozygous <italic>Cx47M282T</italic>
mice, additionally deprived of connexin32, which is also expressed in oligodendrocytes, died within six weeks after birth and displayed severe myelin defects accompanied by astrogliosis and activated microglia. These results strongly suggest that PMLD1 is caused by the loss of Cx47 channel function that results in impaired panglial coupling in white matter tissue.</p>
</abstract>
<abstract abstract-type="summary"><title>Author Summary</title>
<p>Oligodendrocytes are the myelinating cells of the central nervous system. Together with astrocytes, oligodendrocytes form networks of coupled glial cells—so-called panglial networks—which are built by gap junctions, i.e. intercellular channels composed of connexin proteins. Pelizaeus-Merzbacher–like disease is an inherited early onset myelin disorder of the central nervous system. Certain mutations of the connexin47 gene, which is expressed by oligodendrocytes, cause this disease. Although the course of the human disease is conspicuous and progressive, connexin47 null mice do not show obvious phenotypic alterations suggesting that the disease may be caused by gain of detrimental function due to the connexin47 mutations. Here we introduced a missense mutation that was found in Pelizaeus-Merzbacher–like disease patients into the connexin47 mouse gene. Expression of the mutant connexin47 gene in oligodendrocytes resulted in myelin malformations in young mice but to a relatively mild extent. From a comparison of connexin47 null and connexin47 mutant mice, we conclude that the human Pelizaeus-Merzbacher–like disease is caused by loss of gap junctional coupling, which results in a decreased number of cells coupled within glial networks, and not by a gain of detrimental function of the mutated protein.</p>
</abstract>
<counts><page-count count="20"></page-count>
</counts>
</article-meta>
</front>
</pmc>
<affiliations><list><country><li>Allemagne</li>
<li>France</li>
</country>
<region><li>Berlin</li>
<li>District de Cologne</li>
<li>Rhénanie-du-Nord-Westphalie</li>
<li>Île-de-France</li>
</region>
<settlement><li>Berlin</li>
<li>Bonn</li>
<li>Paris</li>
</settlement>
<orgName><li>Université Pierre-et-Marie-Curie</li>
</orgName>
</list>
<tree><country name="Allemagne"><region name="Rhénanie-du-Nord-Westphalie"><name sortKey="Tress, Oliver" sort="Tress, Oliver" uniqKey="Tress O" first="Oliver" last="Tress">Oliver Tress</name>
</region>
<name sortKey="Degen, Joachim" sort="Degen, Joachim" uniqKey="Degen J" first="Joachim" last="Degen">Joachim Degen</name>
<name sortKey="Dicke, Nikolai" sort="Dicke, Nikolai" uniqKey="Dicke N" first="Nikolai" last="Dicke">Nikolai Dicke</name>
<name sortKey="Hartmann, Dieter" sort="Hartmann, Dieter" uniqKey="Hartmann D" first="Dieter" last="Hartmann">Dieter Hartmann</name>
<name sortKey="Kettenmann, Helmut" sort="Kettenmann, Helmut" uniqKey="Kettenmann H" first="Helmut" last="Kettenmann">Helmut Kettenmann</name>
<name sortKey="Maglione, Marta" sort="Maglione, Marta" uniqKey="Maglione M" first="Marta" last="Maglione">Marta Maglione</name>
<name sortKey="May, Dennis" sort="May, Dennis" uniqKey="May D" first="Dennis" last="May">Dennis May</name>
<name sortKey="Willecke, Klaus" sort="Willecke, Klaus" uniqKey="Willecke K" first="Klaus" last="Willecke">Klaus Willecke</name>
<name sortKey="Zlomuzica, Armin" sort="Zlomuzica, Armin" uniqKey="Zlomuzica A" first="Armin" last="Zlomuzica">Armin Zlomuzica</name>
</country>
<country name="France"><region name="Île-de-France"><name sortKey="Dere, Ekrem" sort="Dere, Ekrem" uniqKey="Dere E" first="Ekrem" last="Dere">Ekrem Dere</name>
</region>
</country>
</tree>
</affiliations>
</record>
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