Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function.
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DOI：
10.1371/journal.pone.0043857
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发表时间：
2012
期刊：
影响因子：
3.7
通讯作者：
Ohazama A
中科院分区：
文献类型：
--
作者：
Oommen S;Francois M;Kawasaki M;Murrell M;Kawasaki K;Porntaveetus T;Ghafoor S;Young NJ;Okamatsu Y;McGrath J;Koopman P;Sharpe PT;Ohazama A
半粒体由复杂的蛋白质网络组成，是上皮组织完整性必不可少的附着装置。破坏会导致水泡性疾病，如大疱性表皮松解症。Sox基因家族的成员在人类和小鼠的发育过程中表现出动态和多样化的表达模式，突变分析提供了证据，表明它们在发育和人类疾病中发挥着显著的多种作用。先前的研究已经证实，小鼠突变体ragge - possum （Raop）表达SOX18转录因子的显性阴性形式，干扰野生型SOX18和相关soxf亚群蛋白SOX7和- 17的功能。本研究表明，纯合子Raop小鼠的皮肤和口腔黏膜上皮从下层间充质组织中广泛脱离，这是由于半底酶破坏导致质膜上方上皮细胞撕裂造成的。此外，在Raop小鼠中发现了几种半脂体蛋白表达失调。我们的数据表明，SOXF转录因子在调节细胞质斑块蛋白组装的形成中发挥作用，破坏SOXF功能导致大疱样表皮松解症皮肤表型。
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.
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