Ishigatsubo Y, Steinberg A D, Klinman D M
Cellular Immunology Section, NINCDS, Bethesda, MD 20892.
Eur J Immunol. 1988 Jul;18(7):1089-93. doi: 10.1002/eji.1830180718.
Systemic autoimmune disease in humans and mice is characterized by hypergammaglobulinemia and abnormally high levels of serum autoantibodies. The presence of certain genes, such as the lpr and gld genes, induces otherwise normal mice to spontaneously develop systemic autoimmunity. To better understand the effect of these genes on the development of hypergammaglobulinemia, we quantitated the absolute number of splenic B cells producing antibodies reactive with each of four autoantigens and two conventional antigens and compared this to the total number of Ig-secreting spleen cells present in these mice. Whereas autoimmune mice had significantly greater numbers of autoantibody-secreting spleen cells than normal mice, they also had significantly greater numbers producing antibodies of conventional specificity. When expressed as a proportion of the total repertoire, no bias towards autoantibody production was present when autoimmune lpr and gld animals were compared to their congenic nonautoimmune C57BL/6 and C3H/HeJ counterparts. We also examined the B cell repertoires of recombinant inbred mice derived by mating autoimmune NZB with normal NFS mice. Some recombinant inbred (RI) lines developed hypergammaglobulinemia and produced large quantities of autoantibody. While evidence for specific (auto)antigenic stimulation was present in some RI lines, hypergammaglobulinemia was commonly associated with polyclonal B cell activation in these autoimmune mice as well.
人类和小鼠的系统性自身免疫疾病的特征是高球蛋白血症和血清自身抗体水平异常升高。某些基因的存在,如lpr和gld基因,会诱导原本正常的小鼠自发地发展出系统性自身免疫。为了更好地理解这些基因对高球蛋白血症发展的影响,我们对产生与四种自身抗原和两种传统抗原反应的抗体的脾B细胞的绝对数量进行了定量,并将其与这些小鼠中存在的分泌Ig的脾细胞总数进行了比较。自身免疫小鼠分泌自身抗体的脾细胞数量明显多于正常小鼠,它们产生具有传统特异性抗体的细胞数量也明显更多。当以总库的比例表示时,将自身免疫的lpr和gld动物与其同基因的非自身免疫C57BL/6和C3H/HeJ对应物进行比较时,不存在对自身抗体产生的偏向。我们还检查了通过将自身免疫的NZB与正常的NFS小鼠交配而获得的重组近交系小鼠的B细胞库。一些重组近交(RI)系出现了高球蛋白血症并产生了大量自身抗体。虽然在一些RI系中存在特异性(自身)抗原刺激的证据,但在这些自身免疫小鼠中,高球蛋白血症通常也与多克隆B细胞活化有关。