Very D L, Panka D J, Weissman D, Wysocki L, Manser T, Marshak-Rothstein A
Department of Microbiology, Boston University School of Medicine, Massachusetts 02118.
Immunology. 1993 Dec;80(4):518-26.
It has been proposed that the autoantibody-secreting cells active during autoimmune diseases are derived from B cells initially responding to environmental antigens. In order to test the relationship between the antigen-induced and autoimmune repertoires, we monitored the fate of antigen-activated idiotypically defined B cells present in mice that developed the systemic lupus erythematosus (SLE)-like syndrome associated with the lpr mutation. Mice homozygous for both the A/J-derived Igh and Ig kappa region haplotypes and the lpr mutation were bred. Immunization of these mice with p-azophenylarsonate (Ars)-protein conjugates elicited the idiotypic components (IdCR) characteristic of the A/J anti-Ars response and did not interfere with the spontaneous development of the lpr-mediated autoimmune disease. These Id/lpr mice provided an ideal system for studying the relationship between the exogenously and endogenously induced responses because: (1) VHIdCR antibodies have been shown to bind autoantigens in vitro; and (2) serological and molecular reagents exist which can identify and monitor VHIdCR antibody production as disease progresses. Serum samples and hybridoma cell lines derived from non-immune as well as Ars-keyhole limpet haemocyanin (KLH)-immunized Id/lpr mice were monitored for idiotype expression as well as Ars and ssDNA reactivity at various stages of disease progression. We found that antibodies utilizing the VHIdCR gene segment did not preferentially contribute to the autoantibody pool. Moreover, even when IdCR B-cell clones were expanded by deliberate immunization with Ars-KLH, Ars non-binding variants were only rarely detected among the activated B-cell populations of diseased mice. These results indicate that there is only minimal overlap between the VHIdCR conventional and autoimmune repertoires.
有人提出,自身免疫性疾病期间活跃的自身抗体分泌细胞源自最初对环境抗原产生反应的B细胞。为了测试抗原诱导的和自身免疫的抗原受体库之间的关系,我们监测了存在于发生与lpr突变相关的系统性红斑狼疮(SLE)样综合征的小鼠体内的抗原激活的、独特型定义的B细胞的命运。培育出同时具有源自A/J的Igh和Ig κ区域单倍型以及lpr突变的纯合小鼠。用对氨基苯砷酸盐(Ars)-蛋白质偶联物免疫这些小鼠,引发了A/J抗Ars反应特有的独特型成分(IdCR),并且不干扰lpr介导的自身免疫性疾病的自发发展。这些Id/lpr小鼠为研究外源性和内源性诱导反应之间的关系提供了一个理想的系统,因为:(1)VHIdCR抗体已被证明在体外能结合自身抗原;(2)存在血清学和分子试剂,可在疾病进展过程中识别和监测VHIdCR抗体的产生。监测了来自非免疫以及Ars-钥孔血蓝蛋白(KLH)免疫的Id/lpr小鼠的血清样本和杂交瘤细胞系在疾病进展的各个阶段的独特型表达以及对Ars和单链DNA的反应性。我们发现利用VHIdCR基因片段的抗体并非优先进入自身抗体库。此外,即使通过用Ars-KLH进行刻意免疫使IdCR B细胞克隆扩增,在患病小鼠的活化B细胞群体中也仅很少检测到不结合Ars的变体。这些结果表明,VHIdCR传统抗原受体库和自身免疫抗原受体库之间只有最小程度的重叠。