Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.

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...

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Main Authors: Shelly Oommen, Mathias Francois, Maiko Kawasaki, Melanie Murrell, Katsushige Kawasaki, Thantrira Porntaveetus, Sarah Ghafoor, Neville J Young, Yoshimasa Okamatsu, John McGrath, Peter Koopman, Paul T Sharpe, Atsushi Ohazama
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2012-01-01
Series:PLoS ONE
Online Access:https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0043857&type=printable
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author Shelly Oommen
Mathias Francois
Maiko Kawasaki
Melanie Murrell
Katsushige Kawasaki
Thantrira Porntaveetus
Sarah Ghafoor
Neville J Young
Yoshimasa Okamatsu
John McGrath
Peter Koopman
Paul T Sharpe
Atsushi Ohazama
author_facet Shelly Oommen
Mathias Francois
Maiko Kawasaki
Melanie Murrell
Katsushige Kawasaki
Thantrira Porntaveetus
Sarah Ghafoor
Neville J Young
Yoshimasa Okamatsu
John McGrath
Peter Koopman
Paul T Sharpe
Atsushi Ohazama
author_sort Shelly Oommen
collection DOAJ
description 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 (Ra(op)) 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 Ra(op) 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 Ra(op) 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.
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institution Kabale University
issn 1932-6203
language English
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publisher Public Library of Science (PLoS)
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series PLoS ONE
spelling doaj-art-eaafc9ef60df4eba99720c82ba5fda462025-08-20T03:26:35ZengPublic Library of Science (PLoS)PLoS ONE1932-62032012-01-0179e4385710.1371/journal.pone.0043857Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.Shelly OommenMathias FrancoisMaiko KawasakiMelanie MurrellKatsushige KawasakiThantrira PorntaveetusSarah GhafoorNeville J YoungYoshimasa OkamatsuJohn McGrathPeter KoopmanPaul T SharpeAtsushi OhazamaHemidesmosomes 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 (Ra(op)) 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 Ra(op) 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 Ra(op) 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.https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0043857&type=printable
spellingShingle Shelly Oommen
Mathias Francois
Maiko Kawasaki
Melanie Murrell
Katsushige Kawasaki
Thantrira Porntaveetus
Sarah Ghafoor
Neville J Young
Yoshimasa Okamatsu
John McGrath
Peter Koopman
Paul T Sharpe
Atsushi Ohazama
Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
PLoS ONE
title Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
title_full Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
title_fullStr Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
title_full_unstemmed Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
title_short Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
title_sort cytoplasmic plaque formation in hemidesmosome development is dependent on soxf transcription factor function
url https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0043857&type=printable
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