The FSHD Atrophic Myotube Phenotype Is Caused by DUX4 Expression
Identifieur interne : 002A75 ( Pmc/Curation ); précédent : 002A74; suivant : 002A76The FSHD Atrophic Myotube Phenotype Is Caused by DUX4 Expression
Auteurs : Céline Vanderplanck [Belgique] ; Eugénie Ansseau [Belgique] ; Sébastien Charron [Belgique] ; Nadia Stricwant [Belgique] ; Alexandra Tassin [Belgique] ; Dalila Laoudj-Chenivesse [France] ; Steve D. Wilton [Australie] ; Frédérique Coppée [Belgique] ; Alexandra Belayew [Belgique]Source :
- PLoS ONE [ 1932-6203 ] ; 2011.
Abstract
Facioscapulohumeral muscular dystrophy (FSHD) is linked to deletions in 4q35 within the
We now show that transfection of myoblasts with a
These results constitute a proof of concept for the development of therapeutic approaches for FSHD targeting
Url:
DOI: 10.1371/journal.pone.0026820
PubMed: 22053214
PubMed Central: 3203905
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">The FSHD Atrophic Myotube Phenotype Is Caused by DUX4 Expression</title>
<author><name sortKey="Vanderplanck, Celine" sort="Vanderplanck, Celine" uniqKey="Vanderplanck C" first="Céline" last="Vanderplanck">Céline Vanderplanck</name>
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<author><name sortKey="Belayew, Alexandra" sort="Belayew, Alexandra" uniqKey="Belayew A" first="Alexandra" last="Belayew">Alexandra Belayew</name>
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<front><div type="abstract" xml:lang="en"><sec><title>Background</title>
<p>Facioscapulohumeral muscular dystrophy (FSHD) is linked to deletions in 4q35 within the <italic>D4Z4</italic>
repeat array in which we identified the <underline>d</underline>
o<underline>u</underline>
ble homeobo<underline>x</underline>
4 (<italic>DUX4)</italic>
gene. We found stable DUX4 mRNAs only derived from the most distal <italic>D4Z4</italic>
unit and unexpectedly extended to the flanking <italic>pLAM</italic>
region that provided an intron and a polyadenylation signal. <italic>DUX4</italic>
encodes a transcription factor expressed in FSHD but not control primary myoblasts or muscle biopsies. The DUX4 protein initiates a large transcription deregulation cascade leading to muscle atrophy and oxidative stress, which are FSHD key features.</p>
</sec>
<sec><title>Methodology/Principal Findings</title>
<p>We now show that transfection of myoblasts with a <italic>DUX4</italic>
expression vector leads to atrophic myotube formation associated with the induction of E3 ubiquitin ligases (MuRF1 and Atrogin1/MAFbx) typical of muscle atrophy. DUX4 induces expression of downstream targets deregulated in FSHD such as mu-crystallin and TP53. We developed specific siRNAs and antisense oligonucleotides (AOs) targeting the <italic>DUX4</italic>
mRNA. Addition of these antisense agents to primary FSHD myoblast cultures suppressed DUX4 protein expression and affected expression of the above-mentioned markers.</p>
</sec>
<sec><title>Conclusions/Significance</title>
<p>These results constitute a proof of concept for the development of therapeutic approaches for FSHD targeting <italic>DUX4</italic>
expression.</p>
</sec>
</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 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">22053214</article-id>
<article-id pub-id-type="pmc">3203905</article-id>
<article-id pub-id-type="publisher-id">PONE-D-11-14845</article-id>
<article-id pub-id-type="doi">10.1371/journal.pone.0026820</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>Genetics</subject>
<subj-group><subject>Gene Expression</subject>
<subj-group><subject>DNA transcription</subject>
<subject>Protein Translation</subject>
<subject>RNA interference</subject>
<subject>RNA processing</subject>
<subject>RNA stability</subject>
</subj-group>
</subj-group>
<subj-group><subject>Molecular Genetics</subject>
<subj-group><subject>Gene Regulation</subject>
</subj-group>
</subj-group>
<subj-group><subject>Gene Function</subject>
<subject>Gene Splicing</subject>
<subject>Genetics of Disease</subject>
</subj-group>
</subj-group>
<subj-group><subject>Molecular Cell Biology</subject>
<subj-group><subject>Cellular Types</subject>
<subj-group><subject>Muscle Fibers</subject>
<subject>Muscle Cells</subject>
</subj-group>
</subj-group>
<subj-group><subject>Gene Expression</subject>
<subj-group><subject>Protein Translation</subject>
<subject>RNA interference</subject>
<subject>RNA processing</subject>
<subject>RNA stability</subject>
</subj-group>
</subj-group>
<subj-group><subject>Cell Growth</subject>
</subj-group>
</subj-group>
</subj-group>
</article-categories>
<title-group><article-title>The FSHD Atrophic Myotube Phenotype Is Caused by DUX4 Expression</article-title>
<alt-title alt-title-type="running-head">DUX4 Expression in FSHD</alt-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Vanderplanck</surname>
<given-names>Céline</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Ansseau</surname>
<given-names>Eugénie</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Charron</surname>
<given-names>Sébastien</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Stricwant</surname>
<given-names>Nadia</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Tassin</surname>
<given-names>Alexandra</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Laoudj-Chenivesse</surname>
<given-names>Dalila</given-names>
</name>
<xref ref-type="aff" rid="aff2"><sup>2</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Wilton</surname>
<given-names>Steve D.</given-names>
</name>
<xref ref-type="aff" rid="aff3"><sup>3</sup>
</xref>
</contrib>
<contrib contrib-type="author" equal-contrib="yes"><name><surname>Coppée</surname>
<given-names>Frédérique</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author" equal-contrib="yes"><name><surname>Belayew</surname>
<given-names>Alexandra</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>Laboratory of Molecular Biology, University of Mons, Mons, Belgium</addr-line>
</aff>
<aff id="aff2"><label>2</label>
<addr-line>INSERM U1046, Le Centre Hospitalier Universitaire Arnaud de Villeneuve, Montpellier, France</addr-line>
</aff>
<aff id="aff3"><label>3</label>
<addr-line>Molecular Genetic Therapy Group, University of Western Australia, Nedlands, Australia</addr-line>
</aff>
<contrib-group><contrib contrib-type="editor"><name><surname>Chadwick</surname>
<given-names>Brian P.</given-names>
</name>
<role>Editor</role>
<xref ref-type="aff" rid="edit1"></xref>
</contrib>
</contrib-group>
<aff id="edit1">Florida State University, United States of America</aff>
<author-notes><corresp id="cor1">* E-mail: <email>alexandra.belayew@umons.ac.be</email>
</corresp>
<fn fn-type="con"><p>Conceived and designed the experiments: SDW FC AB. Performed the experiments: CV EA SC NS AT. Analyzed the data: CV EA SW FC AB. Contributed reagents/materials/analysis tools: AT DL SDW. Wrote the paper: CV FC SDW FC AB.</p>
</fn>
</author-notes>
<pub-date pub-type="collection"><year>2011</year>
</pub-date>
<pub-date pub-type="epub"><day>28</day>
<month>10</month>
<year>2011</year>
</pub-date>
<volume>6</volume>
<issue>10</issue>
<elocation-id>e26820</elocation-id>
<history><date date-type="received"><day>1</day>
<month>8</month>
<year>2011</year>
</date>
<date date-type="accepted"><day>3</day>
<month>10</month>
<year>2011</year>
</date>
</history>
<permissions><copyright-statement>Vanderplanck et al. 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 credited.</copyright-statement>
<copyright-year>2011</copyright-year>
</permissions>
<abstract><sec><title>Background</title>
<p>Facioscapulohumeral muscular dystrophy (FSHD) is linked to deletions in 4q35 within the <italic>D4Z4</italic>
repeat array in which we identified the <underline>d</underline>
o<underline>u</underline>
ble homeobo<underline>x</underline>
4 (<italic>DUX4)</italic>
gene. We found stable DUX4 mRNAs only derived from the most distal <italic>D4Z4</italic>
unit and unexpectedly extended to the flanking <italic>pLAM</italic>
region that provided an intron and a polyadenylation signal. <italic>DUX4</italic>
encodes a transcription factor expressed in FSHD but not control primary myoblasts or muscle biopsies. The DUX4 protein initiates a large transcription deregulation cascade leading to muscle atrophy and oxidative stress, which are FSHD key features.</p>
</sec>
<sec><title>Methodology/Principal Findings</title>
<p>We now show that transfection of myoblasts with a <italic>DUX4</italic>
expression vector leads to atrophic myotube formation associated with the induction of E3 ubiquitin ligases (MuRF1 and Atrogin1/MAFbx) typical of muscle atrophy. DUX4 induces expression of downstream targets deregulated in FSHD such as mu-crystallin and TP53. We developed specific siRNAs and antisense oligonucleotides (AOs) targeting the <italic>DUX4</italic>
mRNA. Addition of these antisense agents to primary FSHD myoblast cultures suppressed DUX4 protein expression and affected expression of the above-mentioned markers.</p>
</sec>
<sec><title>Conclusions/Significance</title>
<p>These results constitute a proof of concept for the development of therapeutic approaches for FSHD targeting <italic>DUX4</italic>
expression.</p>
</sec>
</abstract>
<counts><page-count count="14"></page-count>
</counts>
</article-meta>
</front>
</pmc>
</record>
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