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

Oommen, Shelly, Francois, Mathias, Kawasaki, Maiko, Murrell, Melanie, Kawasaki, Katsushige, Porntaveetus, Thantrira, Ghafoor, Sarah, Young, Neville J., Okamatsu, Yoshimasa, McGrath, John, Koopman, Peter, Sharpe, Paul T. and Ohazama, Atsushi (2012) Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function. PloS One, 7 9: . doi:10.1371/journal.pone.0043857


Author Oommen, Shelly
Francois, Mathias
Kawasaki, Maiko
Murrell, Melanie
Kawasaki, Katsushige
Porntaveetus, Thantrira
Ghafoor, Sarah
Young, Neville J.
Okamatsu, Yoshimasa
McGrath, John
Koopman, Peter
Sharpe, Paul T.
Ohazama, Atsushi
Title Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function
Journal name PloS One   Check publisher's open access policy
ISSN 1932-6203
Publication date 2012-09-04
Year available 2012
Sub-type Article (original research)
DOI 10.1371/journal.pone.0043857
Open Access Status DOI
Volume 7
Issue 9
Total pages 9
Place of publication San Francisco, CA, United States
Publisher Public Library of Science
Language eng
Subject 1300 Biochemistry, Genetics and Molecular Biology
1100 Agricultural and Biological Sciences
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 (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.
Formatted 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.
Keyword Epidermolysis bullosa simplex
Epidermal basement membrane
Molecular organization
Pyloric atresia
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2013 Collection
Institute for Molecular Bioscience - Publications
 
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