Kasaian M T, Ikematsu H, Balow J E, Casali P
Department of Pathology, New York University School of Medicine, NY 10016.
J Immunol. 1994 Mar 15;152(6):3137-51.
Anti-DNA IgA autoantibodies play an important immunopathologic role in SLE patients. To analyze the cellular origin and the VH and VL structure of anti-DNA IgA autoantibodies, we generated five IgA1 mAbs to DNA using B lymphocytes from three SLE patients. Two mAbs bound to ssDNA only and one to both ssDNA and dsDNA (monoreactive antibodies). The remaining two mAbs bound to DNA (one to ssDNA and the other to both ssDNA and dsDNA) and to other self and foreign Ag (polyreactive antibodies). The IgA mAb relative avidity for DNA ranged from 7.5 x 10(-8) to 8.0 x 10(-10) g/microliters. The anti-DNA IgA mAb used VH segments of the VHI(VI-3b), VHII (VH2-MC2), VHIII (WHG16G and VH26c), and VHIV (V71-2) families in conjunction with V kappa I, V kappa IIIb, or V lambda I segments. All IgA mAb VH segments were juxtaposed with JH4b segments. The heavy chain CDR3 sequences were divergent in composition and length. When compared with those of the closest reported germ line genes, the IgA mAb VH and VL gene sequences displayed a number of differences. That these differences represented somatic point mutations was formally proved in both the monoreactive IgA mAb 412.67.F1.3 and the polyreactive IgA mAb 412.66.F1 VH segments by differential PCR amplification and cloning and sequencing of genomic DNA from the mAb-producing cell lines and autologous polymorphonuclear cells. The sequences of the germ line genes that putatively gave rise to the mAb 412.67.F1.3 and mAb 412.66.F1 VH segments were identical with those of the WHG16G and VH26c genes, respectively. In not only the monoreactive mAb 412.67.F1.3 but also the polyreactive mAb 412.66.F1 and mAb 448.9G.F1 VH segments, the higher concentration of replacement (R) mutations and the higher R:S (silent) mutation ratios in the complementarity-determining region (infinity; 19:0) than in the framework region (1.0) (p = 0.00001, chi 2 test) were highly consistent with selection by Ag. In the five IgA mAb VH and VL segments, the putative and verified somatic point mutations yielded 68 amino acid replacements, of which 38 were nonconserved. Twenty of these yielded positively charged or polar residues that play a major role in DNA binding, including seven Arg, five Lys, three Tyr, two Gln, two His, and a Thr. The conserved amino acid changes included seven Asn. These findings suggest that anti-DNA IgA autoantibodies use a broad selection of VH and VL genes and enhance their fit for Ag by undergoing somatic hypermutation and Ag selection.(ABSTRACT TRUNCATED AT 400 WORDS)
抗DNA IgA自身抗体在系统性红斑狼疮(SLE)患者中发挥着重要的免疫病理作用。为了分析抗DNA IgA自身抗体的细胞来源以及VH和VL结构,我们利用3例SLE患者的B淋巴细胞制备了5种针对DNA的IgA1单克隆抗体(mAb)。2种mAb仅与单链DNA(ssDNA)结合,1种与ssDNA和双链DNA(dsDNA)均结合(单反应性抗体)。其余2种mAb与DNA结合(1种与ssDNA结合,另1种与ssDNA和dsDNA均结合),还与其他自身和外来抗原结合(多反应性抗体)。这些IgA mAb对DNA的相对亲和力范围为7.5×10⁻⁸至8.0×10⁻¹⁰g/微升。抗DNA IgA mAb使用了VHI(VI - 3b)、VHII (VH2 - MC2)、VHIII (WHG16G和VH26c)和VHIV (V71 - 2)家族的VH片段,分别与VκI、VκIIIb或VλI片段结合。所有IgA mAb的VH片段都与JH4b片段并列。重链互补决定区3(CDR3)序列在组成和长度上存在差异。与最接近的已报道种系基因相比,IgA mAb的VH和VL基因序列存在一些差异。通过对产生mAb的细胞系和自体多形核细胞的基因组DNA进行差异PCR扩增、克隆和测序,在单反应性IgA mAb 412.67.F1.3和多反应性IgA mAb 412.66.F1的VH片段中正式证实了这些差异代表体细胞点突变。推测产生mAb 412.67.F1.3和mAb 412.66.F1 VH片段的种系基因序列分别与WHG16G和VH26c基因的序列相同。不仅在单反应性mAb 412.67.F1.3中,而且在多反应性mAb 412.66.F1和mAb 448.9G.F1的VH片段中,互补决定区(无限大;19:0)中替换(R)突变的浓度较高以及R:S(沉默)突变率高于框架区(1.0)(p = 0.00001,卡方检验),这与抗原选择高度一致。在5种IgA mAb的VH和VL片段中,推测和验证的体细胞点突变产生了68个氨基酸替换,其中38个是非保守的。其中20个产生了在DNA结合中起主要作用的带正电荷或极性残基,包括7个精氨酸、5个赖氨酸、3个酪氨酸、2个谷氨酰胺、2个组氨酸和1个苏氨酸。保守的氨基酸变化包括7个天冬酰胺。这些发现表明,抗DNA IgA自身抗体使用多种VH和VL基因,并通过体细胞超突变和抗原选择来增强它们与抗原的契合度。(摘要截短至400字)