大疱性类天疱疮抗BP180免疫球蛋白E自身抗体结合位点的基因定位  

Mapping the binding sites of anti-BP180 immunoglobulin E autoantibodies in bullous pemphigoid

作　　者：Fairley J.A Chang L.F. Giudice G.J. 罗素菊 

机构地区：[1]Department of Dermatology, Medical College of Wisconsin, 8701 Watertown PlankRoad,Milwaukee,WI 53226,United States Dr.

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第9期9-10,共2页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Bullous pemphigoid (BP) is a subepidermal blistering disease characterized by autoantibodies against the hemidesmosomal protein BP180 (BPAg2, type XVII collagen). NC16A, a noncollagenous stretch of the BP180 ectodomain, is the primary target of pathogenic immunoglobulin (Ig)G autoantibodies and IgE class autoantibodies. This study further characterized the IgE-reactive regions of BP180. Of the ten sera from untreated BP patients, eight contained IgE reactive with the entire BP180 ectodomain. The IgE in four of these eight sera reacted with NC16A, whereas in the remaining four sera IgE immunoreactivity was restricted to sites downstream of NC16A. In contrast, IgG reactivity to NC16A was detected in nine of the ten BP sera, and in the remaining serum, IgG, as well as IgE, reacted exclusively with non-NC16A sites on the BP180 ectodomain. Fine mapping of the antigenic sites within NC16A revealed very similar reactivity patterns for IgE and IgG, with NC16A subregion-2 being the major site recognized by both isotypes. Eight of the untreated BP patients were tested for histamine release from their basophils in response to NC16A. Antigenspecific histamine release was observed only in those patients with detectable circulating IgE directed against NC16A (three of eight). Future studies will investigate the pathogenic relevance of anti-BP180 IgE.Bullous pemphigoid （BP） is a subepidermal blistering disease characterized by autoantibodies against the hemidesmosomal protein BP180 （BPAg2, type XVII collagen） . NC16A, a noncollagenous stretch of the BP180 ectodomain, is the primary target of pathogenic immunoglobulin （Ig） G autoantibodies and IgE class autoantibodies. This study further characterized the IgE-reactive regions of BP180. Of the ten sera from untreated BP patients, eight contained IgE reactive with the entire BP180 ectodomain. The IgE in four of these eight sera reacted with NC16A, whereas in the remaining four sera IgE immunoreactivity was restricted to sites downstream of NC16A. In contrast, IgG reactivity to NC16A was detected in nine of the ten BP sera, and in the remaining serum, IgG, as well as IgE, reacted exclusively with non-NC16A sites on the BP180 ectodomain. Fine mapping of the antigenic sites within NC16A revealed very similar reactivity patterns for IgE and IgG, with NC16A subregion-2 being the major site recognized by both isotypes. Eight of the untreated BP patients were tested for histamine release from their basophils in response to NC16A. Antigenspecific histamine release was observed only in those patients with detectable circulating IgE directed against NC16A （three of eight）. Future studies will investigate the pathogenic relevance of anti-BP180 IgE.

关 键 词：大疱性类天疱疮 免疫球蛋白E 自身抗体 结合位点 基因定位 IgE反应 IgG反应 表皮下大疱病 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]