Gilbert D, Brard F, Jovelin F, Tron F
Groupe de Recherche en Immunopathologie (Institut Fédératif de Recherche Multidisciplinaire sur les Peptides, IFR 23), Hôpital Charles Nicolle et Faculté Mixte de Médecine et de Pharmacie, Rouen, France.
J Autoimmun. 1996 Apr;9(2):247-57. doi: 10.1006/jaut.1996.0031.
Systemic lupus erythematosus (SLE) is the prototype of systemic autoimmune diseases. In both human and mouse SLE diseases, the autoimmune response targets a restricted set of autoantigens. Many of them are nucleic acids and proteins involved in the synthesis and processing of DNA or RNA, a characteristic which should be taken into consideration to elucidate the origins of non organ-specific autoantibodies. Several observations, in particular those obtained from experimental models of SLE induced in normal mice, suggest that the breakdown of B-cell tolerance occurs in the periphery. Herewith, we present data further supporting the proposition that SLE-associated autoantibodies originate from natural autoantibody-secreting B cells activated in the internal environment of lupus mice. Thus, one may hypothesize that certain clones of the expanded primary B-cell repertoire are selected to differentiate into harmful IgG autoantibody-secreting clones, thereby raising the question of the nature of immunogenic structures involved in SLE. Our analysis of the immunochemical and structural properties of anti-nucleosome and anti-myeoloperoxidase monoclonal antibodies derived from (NZB x NZW)F1 mice leads us to propose that complexes formed by the association of DNA and DNA-binding proteins and, more generally, by anionic molecules associated with proteins, possess a selective advantage over other autoantigens to induce the differentiation of certain B-cell clones and the very special profile of the SLE-autoimmune response. These DNA/DNA-protein complexes could also play a role in the activation of the T-cell compartment in SLE.
系统性红斑狼疮(SLE)是系统性自身免疫性疾病的典型代表。在人类和小鼠的SLE疾病中,自身免疫反应针对的是一组有限的自身抗原。其中许多是参与DNA或RNA合成与加工的核酸和蛋白质,这一特征在阐明非器官特异性自身抗体的起源时应予以考虑。一些观察结果,特别是从正常小鼠诱导的SLE实验模型中获得的观察结果表明,B细胞耐受性的破坏发生在外周。在此,我们提供的数据进一步支持了SLE相关自身抗体源自狼疮小鼠体内环境中被激活的天然自身抗体分泌B细胞这一观点。因此,可以推测,扩增的初级B细胞库中的某些克隆被选择分化为分泌有害IgG自身抗体的克隆,从而引发了SLE中涉及的免疫原性结构的性质问题。我们对源自(NZB×NZW)F1小鼠的抗核小体和抗髓过氧化物酶单克隆抗体的免疫化学和结构特性的分析使我们提出,由DNA与DNA结合蛋白结合形成的复合物,更普遍地说,由与蛋白质相关的阴离子分子形成的复合物,相对于其他自身抗原有选择性优势,可诱导某些B细胞克隆的分化以及SLE自身免疫反应的特殊特征。这些DNA/DNA - 蛋白质复合物也可能在SLE中T细胞区室的激活中发挥作用。