Iwatsuki K, Harada H, Yokote R, Kaneko F
Department of Dermatology, Fukushima Medical College, Japan.
Br J Dermatol. 1995 Aug;133(2):209-16. doi: 10.1111/j.1365-2133.1995.tb02617.x.
Epidermal desmogleins with molecular weights of 130/140 kDa (Dsg3 or PVA) and 150/160 kDa (Dsg1 or DGI) are recognized by autoantibodies from patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF), respectively. In order to understand the histogenesis of both types of pemphigus, we studied the expression patterns of Dsg1 and Dsg3 during stratification of cultured keratinocytes. Monolayers of cultured normal human keratinocytes demonstrated uniform intercellular staining with PV sera. The staining pattern was distinct from the focal staining with PF sera observed only in the stratified areas. Both Dsg1 and Dsg3 proteins and their mRNA were expressed by the monolayers, whereas no production of Dsg2 (HDGC) mRNA was found. The relative ratio of Dsg3 to the total desmogleins, as determined by density on immunoblotting, decreased as the cultured keratinocytes stratified. In the completely stratified keratinocytes cultured on collagen membrane, Dsg1 became predominant, with subsequent reduction of PV antigen expression. The relative decrease of Dsg3 (PVA) during epidermal differentiation might be responsible for the induction of suprabasal acantholysis in PV.
寻常型天疱疮(PV)患者的自身抗体可识别分子量为130/140 kDa的表皮桥粒芯糖蛋白(Dsg3或PVA),落叶型天疱疮(PF)患者的自身抗体则可识别分子量为150/160 kDa的表皮桥粒芯糖蛋白(Dsg1或DGI)。为了解这两种类型天疱疮的组织发生机制,我们研究了培养的角质形成细胞分层过程中Dsg1和Dsg3的表达模式。培养的正常人角质形成细胞单层经PV血清染色后呈现均匀的细胞间染色。这种染色模式与仅在分层区域观察到的PF血清的局灶性染色不同。单层细胞同时表达Dsg1和Dsg3蛋白及其mRNA,而未发现Dsg2(HDGC)mRNA的产生。通过免疫印迹密度测定,随着培养的角质形成细胞分层,Dsg3与总桥粒芯糖蛋白的相对比例降低。在胶原膜上培养的完全分层的角质形成细胞中,Dsg1占主导地位,随后PV抗原表达减少。表皮分化过程中Dsg3(PVA)的相对减少可能是PV中基底上棘层松解形成的原因。