Serum antibodies and monoclonal antibodies secreted by thymic B-cell clones from patients with myasthenia gravis define striational antigens.
Serum antibodies and monoclonal antibodies secreted by thymic B-cell clones from patients with myasthenia gravis define striational antigens.
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重症肌无力患者胸腺 B 细胞克隆分泌的血清抗体和单克隆抗体定义了纹状体抗原。
DOI：
10.1111/j.1749-6632.1987.tb51290.x
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发表时间：
1987
影响因子：
5.2
通讯作者：
HowardJr,FM
中科院分区：
文献类型：
--
作者：
Williams,CL;Lennon,VA;Momoi,MY;HowardJr,FM
本文应用两例重症肌无力患者的血清抗体和单克隆抗体，对横纹抗体结合的几种抗原进行了生化鉴定。这些单克隆抗体是由从胸腺和胸腺瘤中获得的EB病毒转化的B淋巴细胞克隆分泌的。血清和单克隆抗体与骨骼肌肌节的离散组分反应，产生几种不同的免疫荧光染色模式。免疫印迹分析和酶联免疫吸附试验显示三种不同的抗体特异性：肌球蛋白，α-辅肌动蛋白，和/或肌动蛋白。单个单克隆StrAb与肌动蛋白或肌球蛋白的肌肉和非肌肉同种型反应。值得注意的是，收缩蛋白（1）与质膜中的乙酰胆碱受体（AChR）相关，（2）在转化细胞中发生生化改变。因此，可以想象的是，从胸腺上皮细胞进行肿瘤转化的新抗原AChR相关的收缩蛋白的释放可能提供了免疫原性刺激的生产的StrAb。更精确的定义StrAb特异性MG和/或胸腺瘤的个体患者可能提供这些疾病的诊断和/或预后分类的基础。此外，单克隆抗体将是有用的实验测试的潜在致病性的StrAb。
The biochemical identities of several antigens to which striational antibodies bind were determined by using serum antibodies and monoclonal antibodies from two patients with myasthenia gravis. The monoclonal antibodies were secreted by EBV-transformed B-lymphocyte clones obtained from thymus and thymoma. Serum and monoclonal antibodies reacted with discrete components of the skeletal muscle sarcomere, giving rise to several different patterns of immunofluorescence staining. Immunoblot analyses and enzyme-linked immunosorbent assays revealed three different antibody specificities: myosin, alpha-actinin, and/or actin. Individual monoclonal StrAb reacted with both muscle and nonmuscle isotypes of actin or myosin. It is noteworthy that contractile proteins (1) are associated with acetylcholine receptors (AChR) in plasma membranes, and (2) are biochemically altered in transformed cells. It is therefore conceivable that the release of neoantigenic AChR-associated contractile proteins from thymic epithelial cells undergoing neoplastic transformation may provide the immunogenic stimulus for production of StrAb. More precise definition of StrAb specificities in individual patients with MG and/or thymoma might provide a basis for diagnostic and/or prognostic classification of these diseases. Furthermore, the monoclonal antibodies will be useful in experimentally testing the potential pathogenicity of StrAb.