Strohal R, Krömer G, Wolf H, Wick G, Kofler R
Institut für Allgemeine und Experimentelle Pathologie, Universität Innsbruck.
Wien Klin Wochenschr. 1988 Dec 2;100(23):753-60.
This review summarizes studies from our and other laboratories attempting to define the molecular and clonotypic origin of autoantibodies that typify systemic autoimmune diseases such as lupus and rheumatoid arthritis. Comparative restriction fragment length polymorphism (RFLP) studies investigating the immunoglobulin (Ig) germline gene organization in lupus-prone strains of mice suggested that the disease can develop in different Ig heavy (H) and light (L) chain haplotypes, and that the Ig germline genes in lupus mice are probably normal. Analysis of the Ig gene segments expressed in monoclonal autoantibodies from autoimmune mice revealed that similar, and in some instances even identical, gene segments are expressed in autoantibodies and in antibodies to exogenous antigens, and that anti-self and anti-non-self responses are encoded by the same, or at least an overlapping germline gene repertoire. A large variety of Ig variable (V), diversity (D), and joining (J) gene segments can encode autoantibodies with different specificities, and both germline genes and somatically-mutated genes can be expressed in such antibodies.
本综述总结了我们实验室及其他实验室的研究，这些研究试图确定自身抗体的分子和克隆型起源，这些自身抗体是系统性自身免疫性疾病（如狼疮和类风湿性关节炎）的典型特征。比较限制性片段长度多态性（RFLP）研究调查了狼疮易感小鼠品系中的免疫球蛋白（Ig）种系基因组织，结果表明该疾病可在不同的Ig重链（H）和轻链（L）单倍型中发生，且狼疮小鼠中的Ig种系基因可能是正常的。对来自自身免疫小鼠的单克隆自身抗体中表达的Ig基因片段的分析显示，在自身抗体和针对外源性抗原的抗体中表达了相似的，甚至在某些情况下相同的基因片段，并且抗自身和抗非自身反应由相同的，或至少是重叠的种系基因库编码。各种各样的Ig可变区（V）、多样性区（D）和连接区（J）基因片段可编码具有不同特异性的自身抗体，种系基因和体细胞突变基因均可在这类抗体中表达。
