STRUCTURE AND FUNCTION OF AN AUTOIMMUNE ANTIGEN

循环中针对核抗原的抗体的存在 抗原是许多全身性风湿性关节炎的常见症状， 疾病 许多系统性红斑狼疮（SLE）患者 产生抗体， 核糖核蛋白复合物（snRNP）发现于真核细胞中。 沉淀snRNP的自身免疫血清类型 含有核小RNA U1、U2、U4、U 5和U6的细胞， 称为抗Sm。 由于抗Sm抗体很少产生， 其他结缔组织疾病患者， 这种特异性作为SLE的有用血清学标记。 抗Sm血清识别的蛋白质之一是11，000 道尔顿E蛋白。 这种蛋白质是所有抗- Sm反应性snRNP，但对其结构或 功能 我们计划使用我们最近分离的cDNA克隆， E蛋白来表征这种自身免疫抗原的基因， 并研究这种蛋白质在细胞生理学中的作用。 最后，我们计划利用蛋白质的结构信息 通过对E蛋白基因进行测序来确定 这种蛋白质的一部分实际上被抗Sm 抗体的 这些研究不仅应加强我们对 自身免疫性疾病的病因，但应该帮助我们设计一个 克隆和分析相关Sm的合理策略 抗原

The presence of circulating antibodies directed against nuclear antigens is a common symptom to many systemic rheumatic diseases. Many patients with systemic lupus erythematosus (SLE) produce antibodies which interact with abundant small nuclear ribonucleoprotein complexes (snRNPs) found in eukaryotic cells. The class of autoimmune sera which precipitate snRNPs containing the small nuclear RNAs U1, U2, U4, U5 and U6 is called anti-Sm. Since anti-Sm antibodies are only rarely produced by patients with other connective tissue diseases, the presence of this specificity serves as a useful serological marker for SLE. One of the proteins recognized by anti-Sm sera is the 11,000 dalton "E" protein. This protein is a component of all of the anti- Sm reactive snRNPs, but little is known about its structure or function. We plan to use our recently isolated cDNA clone for the E protein to characterize the genes for this autoimmune antigen, and to investigate the role of this protein in cellular physiology. Finally we plan to exploit the protein structural information obtained from sequencing the E protein gene to determine which portion of this protein is actually recognized by anti-Sm antibodies. These studies should not only enhance our understanding of the etiology of autoimmune disease, but should help us design a rational strategy for the cloning and analysis of related Sm antigens.