The analysis of pathophysiology of autoimmune bullous diseases by using retrovirus vector

寻常天疱疮（PV）是一种自身免疫性大疱性疾病，其循环中存在抗桥粒芯糖蛋白（Dsg）的IgG自身抗体。我们最近建立了Dsg 3特异性T细胞克隆，诱导PV表型和分离的cDNA克隆的T细胞受体（TCR）。在此，我们用这些TCR cDNA逆转录病毒转导野生型CD 4 ^+ T细胞（rvDsg 3 T细胞），以阐明它们在皮肤抗原特异性自身免疫过程中的致病作用。当将rvDsg 3 T细胞与Dsg 3 ^<-/-> B细胞一起转移到Rag 2 ^<-/->小鼠中时，受体小鼠既未显示临床PV表型、抗Dsg 3 IgG产生，也未显示棘层松解的组织学证据，而是发展出鳞状和鳞状皮肤病变。组织学显示界面皮炎仅在皮肤、粘膜和食管中，其中Dsg 3表达。

Pemphigus vulgaris (PV) is an autoimmune bullous disease with circulating IgG autoantibodies against desmogleins (Dsg). We recently established Dsg3-specific T cell clones that induced PV phenotype and isolated cDNA clones of their T cell receptors (TCR). Herein, we retrovirally transduced wild type CD4^+ T cells with these TCR cDNAs (rvDsg3 T cells) to clarify their pathogenic roles for antigen-specific autoimmune processes in skin. When rvDsg3 T cells were transferred with Dsg3^<-/-> B cells into Rag2^<-/-> mice, the recipient mice neither showed clinical PV phenotype, anti-Dsg3 IgG production, nor histological evidence for acantholysis, but instead developed scaly and erythematous skin lesions. Histology revealed interface dermatitis only in skin, mucous membranes and esophagus, where Dsg3 was expressed.