Dmochowski M, Hashimoto T, Chidgey M A, Yue K K, Wilkinson R W, Nishikawa T, Garrod D R
School of Biological Sciences, University of Manchester, UK.
Br J Dermatol. 1995 Oct;133(4):519-25. doi: 10.1111/j.1365-2133.1995.tb02698.x.
We have shown previously that IgG antibodies in certain pemphigus sera, particularly endemic Brazilian pemphigus foliaceus (BPF) sera, react with bovine desmocollins (Dsc), which are transmembranous glycoproteins of desmosome junctions. Desmocollins occur as three different isoforms (Dsc 1, 2 and 3), all of which are represented in the epidermis. In this study, we examined sera of various pemphigus types by immunoblotting purified bovine desmosomes and bovine Dsc 1, 2 and 3 fusion proteins, expressed in pGEX expression vectors. Six of 15 (40.0%) BPF sera, two of 18 (11.1%) non-endemic pemphigus foliaceus sera, eight of 39 (20.5%) pemphigus vulgaris (PV) sera, and two of 11 (18.2%) normal sera, showed reactivity with Dsc from desmosomes. Experiments with fusion proteins showed that no Dsc isoform was specifically recognized by sera of any individual pemphigus type. Our results indicate that the pathogenesis of pemphigus might be more complex than previously believed.
我们之前已经表明，某些天疱疮血清中的IgG抗体，特别是地方性巴西落叶型天疱疮（BPF）血清中的抗体，可与牛桥粒芯蛋白（Dsc）发生反应，牛桥粒芯蛋白是桥粒连接的跨膜糖蛋白。桥粒芯蛋白有三种不同的异构体（Dsc 1、2和3），它们在表皮中均有表达。在本研究中，我们通过免疫印迹法检测了各种类型天疱疮血清，所用抗原为在pGEX表达载体中表达的纯化牛桥粒和牛Dsc 1、2及3融合蛋白。15份BPF血清中的6份（40.0%）、18份非地方性落叶型天疱疮血清中的2份（11.1%）、39份寻常型天疱疮（PV）血清中的8份（20.5%）以及11份正常血清中的2份（18.2%），均显示出与桥粒中的Dsc发生反应。融合蛋白实验表明，任何一种天疱疮类型的血清均未特异性识别某一种Dsc异构体。我们的结果表明，天疱疮的发病机制可能比之前认为的更为复杂。
