Tanaka T, Furukawa F, Imamura S
Department of Dermatology, Faculty of Medicine, Kyoto University, Japan.
J Invest Dermatol. 1994 May;102(5):706-9. doi: 10.1111/1523-1747.ep12374333.
Epidermolysis bullosa acquisita is a subepidermal blistering disease in which patients have autoantibodies against the non-collagenous domain of type VII collagen. Starting with previously isolated 1-kilobase pair (Kb) cDNA for this autoantigen, we isolated overlapping cDNAs with a combined open reading frame of approximately 3.2 Kb, encoding most (approximately 115 kilodaltons [KDa]) of the N-terminal non-collagenous domain of type VII collagen. To localize immunogenic domains, we produced maltose-binding fusion proteins with cDNA encoding different portions of this autoantigen. These cDNA fragments scan from 5' to 3' of this non-collagenous domain and overlap each other. An immunoblot analysis of these fusion proteins with eight epidermolysis bullosa acquisita patient sera demonstrated that each patient serum binds to different regions of this molecule and that epitopes for these patient sera locate throughout this autoantigen. These data suggest that multiple epitopes on the N-terminal non-collagenous domain of type VII collagen are recognized by circulating autoantibodies in patients with epidermolysis bullosa acquisita.
获得性大疱性表皮松解症是一种表皮下疱病，患者体内存在针对VII型胶原非胶原结构域的自身抗体。我们从先前分离得到的针对该自身抗原的1千碱基对（Kb）互补DNA（cDNA）入手，分离出重叠的cDNA，其组合开放阅读框约为3.2 Kb，编码VII型胶原N端非胶原结构域的大部分（约115千道尔顿 [KDa]）。为了定位免疫原性结构域，我们用编码该自身抗原不同部分的cDNA制备了麦芽糖结合融合蛋白。这些cDNA片段从该非胶原结构域的5'端扫描到3'端且相互重叠。用八份获得性大疱性表皮松解症患者血清对这些融合蛋白进行免疫印迹分析表明，每份患者血清都与该分子的不同区域结合，且这些患者血清的表位遍布整个自身抗原。这些数据表明，获得性大疱性表皮松解症患者循环中的自身抗体可识别VII型胶原N端非胶原结构域上的多个表位。
