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Cytoplasmic Plaque Formation in Hemidesmosome Development Is Dependent on SoxF Transcription Factor Function

Identifieur interne : 004397 ( Main/Exploration ); précédent : 004396; suivant : 004398

Cytoplasmic Plaque Formation in Hemidesmosome Development Is Dependent on SoxF Transcription Factor Function

Auteurs : Shelly Oommen [Royaume-Uni] ; Mathias Francois [Australie] ; Maiko Kawasaki [Royaume-Uni] ; Melanie Murrell [Australie] ; Katsushige Kawasaki [Royaume-Uni] ; Thantrira Porntaveetus [Royaume-Uni] ; Sarah Ghafoor [Royaume-Uni] ; Neville J. Young [Australie] ; Yoshimasa Okamatsu [Japon] ; John Mcgrath [Royaume-Uni] ; Peter Koopman [Australie] ; Paul T. Sharpe [Royaume-Uni] ; Atsushi Ohazama [Royaume-Uni, Japon]

Source :

RBID : PMC:3433475

Abstract

Hemidesmosomes are composed of intricate networks of proteins, that are an essential attachment apparatus for the integrity of epithelial tissue. Disruption leads to blistering diseases such as epidermolysis bullosa. Members of the Sox gene family show dynamic and diverse expression patterns during development and mutation analyses in humans and mice provide evidence that they play a remarkable variety of roles in development and human disease. Previous studies have established that the mouse mutant ragged-opossum (Raop) expresses a dominant-negative form of the SOX18 transcription factor that interferes with the function of wild type SOX18 and of the related SOXF-subgroup proteins SOX7 and −17. Here we show that skin and oral mucosa in homozygous Raop mice display extensive detachment of epithelium from the underlying mesenchymal tissue, caused by tearing of epithelial cells just above the plasma membrane due to hemidesmosome disruption. In addition, several hemidesmosome proteins expression were found to be dysregulated in the Raop mice. Our data suggest that SOXF transcription factors play a role in regulating formation of cytoplasmic plaque protein assembly, and that disrupted SOXF function results in epidermolysis bullosa-like skin phenotypes.


Url:
DOI: 10.1371/journal.pone.0043857
PubMed: 22962592
PubMed Central: 3433475


Affiliations:


Links toward previous steps (curation, corpus...)


Le document en format XML

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<p>Hemidesmosomes are composed of intricate networks of proteins, that are an essential attachment apparatus for the integrity of epithelial tissue. Disruption leads to blistering diseases such as epidermolysis bullosa. Members of the
<italic>Sox</italic>
gene family show dynamic and diverse expression patterns during development and mutation analyses in humans and mice provide evidence that they play a remarkable variety of roles in development and human disease. Previous studies have established that the mouse mutant ragged-opossum (
<italic>Ra
<sup>op</sup>
</italic>
) expresses a dominant-negative form of the SOX18 transcription factor that interferes with the function of wild type SOX18 and of the related SOXF-subgroup proteins SOX7 and −17. Here we show that skin and oral mucosa in homozygous
<italic>Ra
<sup>op</sup>
</italic>
mice display extensive detachment of epithelium from the underlying mesenchymal tissue, caused by tearing of epithelial cells just above the plasma membrane due to hemidesmosome disruption. In addition, several hemidesmosome proteins expression were found to be dysregulated in the
<italic>Ra
<sup>op</sup>
</italic>
mice. Our data suggest that SOXF transcription factors play a role in regulating formation of cytoplasmic plaque protein assembly, and that disrupted SOXF function results in epidermolysis bullosa-like skin phenotypes.</p>
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<name sortKey="Hemler, Me" uniqKey="Hemler M">ME Hemler</name>
</author>
</analytic>
</biblStruct>
<biblStruct>
<analytic>
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<name sortKey="Irrthum, A" uniqKey="Irrthum A">A Irrthum</name>
</author>
<author>
<name sortKey="Devriendt, K" uniqKey="Devriendt K">K Devriendt</name>
</author>
<author>
<name sortKey="Chitayat, D" uniqKey="Chitayat D">D Chitayat</name>
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<author>
<name sortKey="Matthijs, G" uniqKey="Matthijs G">G Matthijs</name>
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<author>
<name sortKey="Glade, C" uniqKey="Glade C">C Glade</name>
</author>
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</biblStruct>
<biblStruct>
<analytic>
<author>
<name sortKey="Ohazama, A" uniqKey="Ohazama A">A Ohazama</name>
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<author>
<name sortKey="Johnson, Eb" uniqKey="Johnson E">EB Johnson</name>
</author>
<author>
<name sortKey="Ota, Ms" uniqKey="Ota M">MS Ota</name>
</author>
<author>
<name sortKey="Choi, Hj" uniqKey="Choi H">HJ Choi</name>
</author>
<author>
<name sortKey="Porntaveetus, T" uniqKey="Porntaveetus T">T Porntaveetus</name>
</author>
</analytic>
</biblStruct>
<biblStruct>
<analytic>
<author>
<name sortKey="Chiba, H" uniqKey="Chiba H">H Chiba</name>
</author>
<author>
<name sortKey="Gotoh, T" uniqKey="Gotoh T">T Gotoh</name>
</author>
<author>
<name sortKey="Kojima, T" uniqKey="Kojima T">T Kojima</name>
</author>
<author>
<name sortKey="Satohisa, S" uniqKey="Satohisa S">S Satohisa</name>
</author>
<author>
<name sortKey="Kikuchi, K" uniqKey="Kikuchi K">K Kikuchi</name>
</author>
</analytic>
</biblStruct>
<biblStruct>
<analytic>
<author>
<name sortKey="Spagnuolo, R" uniqKey="Spagnuolo R">R Spagnuolo</name>
</author>
<author>
<name sortKey="Corada, M" uniqKey="Corada M">M Corada</name>
</author>
<author>
<name sortKey="Orsenigo, F" uniqKey="Orsenigo F">F Orsenigo</name>
</author>
<author>
<name sortKey="Zanetta, L" uniqKey="Zanetta L">L Zanetta</name>
</author>
<author>
<name sortKey="Deuschle, U" uniqKey="Deuschle U">U Deuschle</name>
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<list>
<country>
<li>Australie</li>
<li>Japon</li>
<li>Royaume-Uni</li>
</country>
<region>
<li>Angleterre</li>
<li>Grand Londres</li>
<li>Région de Kantō</li>
</region>
<settlement>
<li>Londres</li>
<li>Tokyo</li>
</settlement>
</list>
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<country name="Royaume-Uni">
<region name="Angleterre">
<name sortKey="Oommen, Shelly" sort="Oommen, Shelly" uniqKey="Oommen S" first="Shelly" last="Oommen">Shelly Oommen</name>
</region>
<name sortKey="Ghafoor, Sarah" sort="Ghafoor, Sarah" uniqKey="Ghafoor S" first="Sarah" last="Ghafoor">Sarah Ghafoor</name>
<name sortKey="Kawasaki, Katsushige" sort="Kawasaki, Katsushige" uniqKey="Kawasaki K" first="Katsushige" last="Kawasaki">Katsushige Kawasaki</name>
<name sortKey="Kawasaki, Maiko" sort="Kawasaki, Maiko" uniqKey="Kawasaki M" first="Maiko" last="Kawasaki">Maiko Kawasaki</name>
<name sortKey="Mcgrath, John" sort="Mcgrath, John" uniqKey="Mcgrath J" first="John" last="Mcgrath">John Mcgrath</name>
<name sortKey="Ohazama, Atsushi" sort="Ohazama, Atsushi" uniqKey="Ohazama A" first="Atsushi" last="Ohazama">Atsushi Ohazama</name>
<name sortKey="Porntaveetus, Thantrira" sort="Porntaveetus, Thantrira" uniqKey="Porntaveetus T" first="Thantrira" last="Porntaveetus">Thantrira Porntaveetus</name>
<name sortKey="Sharpe, Paul T" sort="Sharpe, Paul T" uniqKey="Sharpe P" first="Paul T." last="Sharpe">Paul T. Sharpe</name>
</country>
<country name="Australie">
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<name sortKey="Francois, Mathias" sort="Francois, Mathias" uniqKey="Francois M" first="Mathias" last="Francois">Mathias Francois</name>
</noRegion>
<name sortKey="Koopman, Peter" sort="Koopman, Peter" uniqKey="Koopman P" first="Peter" last="Koopman">Peter Koopman</name>
<name sortKey="Murrell, Melanie" sort="Murrell, Melanie" uniqKey="Murrell M" first="Melanie" last="Murrell">Melanie Murrell</name>
<name sortKey="Young, Neville J" sort="Young, Neville J" uniqKey="Young N" first="Neville J." last="Young">Neville J. Young</name>
</country>
<country name="Japon">
<region name="Région de Kantō">
<name sortKey="Okamatsu, Yoshimasa" sort="Okamatsu, Yoshimasa" uniqKey="Okamatsu Y" first="Yoshimasa" last="Okamatsu">Yoshimasa Okamatsu</name>
</region>
<name sortKey="Ohazama, Atsushi" sort="Ohazama, Atsushi" uniqKey="Ohazama A" first="Atsushi" last="Ohazama">Atsushi Ohazama</name>
</country>
</tree>
</affiliations>
</record>

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