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Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function
Shelly Oommen1, Mathias Francois, Maiko Kawasaki
1Craniofacial Development and Stem Cell Biology, and Biomedical Research Centre, Dental Institute, King's College London, London, United Kingdom.
Plos One
|September 11, 2012
Summary
SOXF transcription factors are crucial for epithelial tissue integrity. Disrupted SOXF function in mice leads to hemidesmosome disruption and epidermolysis bullosa-like skin conditions.
Area of Science:
- Molecular Biology
- Developmental Biology
- Genetics
Background:
- Hemidesmosomes are vital protein complexes for epithelial tissue attachment.
- Disruption of hemidesmosomes causes blistering diseases like epidermolysis bullosa.
- SOXF transcription factors (SOX7, SOX17, SOX18) have diverse developmental roles.
Purpose of the Study:
- To investigate the role of SOXF transcription factors in hemidesmosome formation and epithelial integrity.
- To analyze the phenotype of the ragged-opossum (Ra(op)) mouse model with disrupted SOXF function.
Main Methods:
- Analysis of skin and oral mucosa in homozygous Ra(op) mice.
- Examination of hemidesmosome structure and protein expression.
- Investigating the effects of dominant-negative SOX18 on SOXF subgroup proteins.
Main Results:
- Homozygous Ra(op) mice exhibit extensive epithelial detachment due to hemidesmosome disruption.
- Dysregulation of several hemidesmosome protein expressions was observed in Ra(op) mice.
- The Ra(op) mutation causes tearing of epithelial cells just above the plasma membrane.
Conclusions:
- SOXF transcription factors are implicated in regulating the assembly of cytoplasmic plaque proteins in hemidesmosomes.
- Disrupted SOXF function leads to epidermolysis bullosa-like phenotypes.
- This study highlights a novel role for SOXF proteins in maintaining epithelial adhesion.