Antibodies to the amino-terminal domain of desmoglein 1 are retained during transition from pemphigus vulgaris to pemphigus foliaceus
Antibodies to the amino-terminal domain of desmoglein 1 are retained during transition from pemphigus vulgaris to pemphigus foliaceus
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DOI：
10.1684/ejd.2014.2277
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发表时间：
2014-03-01
影响因子：
2.5
通讯作者：
Hashimoto, Takashi
中科院分区：
文献类型：
--
作者：
Espana, Agustin;Koga, Hiroshi;Hashimoto, Takashi
背景：寻常型天疱疮(PV)和叶型天疱疮(Pf)是由抗桥粒蛋白3(Dsg3)和/或DSG1抗体介导的两种水疱性皮肤病。从PV到Pf的表型转变很少有报道。目的：研究在这一过渡过程中，双链球蛋白对胞外(EC)结构域的免疫应答。材料和方法：我们报告了两名PV患者，他们随后发展为PF表型。为了绘制这些情况下的构象表位图，我们通过免疫沉淀-免疫印迹分析检测了两名患者血清的反应性，使用了Dsg2骨骼上的五个DSG1/Dsg3结构域互换分子。结果：在PV期和PF期，DSG1的EC1结构域的特异性反应性保持不变。Dsg3的EC1区和EC2区与缓解期和PF期的血清反应较弱。结论：我们的研究结果表明，DSG1的EC区的氨基端致病抗体保留，而Dsg3在从PV向PF的转变过程中发生了相当大的表位变化，Dsg3的EC1区的致病抗体绝对或显著减少。
Background: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are two blistering skin diseases mediated by antibodies to desmoglein 3 (Dsg3) and/or Dsg1. Phenotypic transition from PV to PF is rarely reported. Objectives: To determine the immune response to extracellular (EC) domains of Dsgs during this transition. Materials and Methods: We report two PV patients who subsequently developed a PF phenotype. To map the conformational epitopes in these cases, we examined the reactivity of the sera of two patients by immunoprecipitation-immunoblotting analysis, using five Dsg1/Dsg3 domain-swapped molecules on a back-bone of Dsg2. Results: Reactivity exclusively with the EC1 domain of Dsg1 was maintained in both PV and PF stages. No reactivity to Dsg3 in the PF stage was found in patient 1. Various changes in immunoreactivity to Dsg3 were found and the EC1 and EC2 domains of Dsg3 reacted weakly to serum taken at remission and PF stages in patient 2. Conclusions: Our findings suggest that amino-terminal pathogenic antibodies to the EC domain of Dsg1 were retained, while considerable epitope changes occurred in response to Dsg3 during the shift from PV to PF, with an absolute or significant decrease in pathogenic antibodies to the EC1 domain of Dsg3.