Painter C J, Monestier M, Chew A, Bona-Dimitriu A, Kasturi K, Bailey C, Scott V E, Sidman C L, Bona C A
Department of Microbiology, Mount Sinai School of Medicine, New York, New York 10029.
J Exp Med. 1988 Mar 1;167(3):1137-53. doi: 10.1084/jem.167.3.1137.
Several hundred hybridomas were obtained from 1-2-mo-old viable motheaten (mev) mice. Among the Ig-secreting hybridomas tested, greater than 50% (17/33) exhibited reactivity for autoantigens, supporting the idea that the Ly-1 B cells that predominate in mev mice contain frequent precursors of autoantibody-forming cells. Certain of the specificities of these autoantibodies correlated with the documented pathophysiology of mev mice (antithymocyte, -erythrocyte, -skin, -kidney, and -IgG); others were specific for autoantigens not previously observed in motheaten mice but though to be involved in other autoimmune diseases (e.g., intrinsic factor, transferrin, myelin basic protein, and thyroglobulin). About 2 of 3 (11/17) of the self-reactive antibodies exhibited multispecific binding activity for various autoantigens. Analysis by Northern blotting of the V gene families used in mev autoantibodies showed a random usage of VH families and a biased usage of four Vk gene families. Of 16 autoantibodies tested, 12 used a Vk gene from the Vk1, 4, 10, or 19 families. These patterns of Vk gene usage differ from nonautoimmune control animals. Overall, an immunoregulatory defect operating at a more generalized level than the VH or Vk loci, and due to a single gene mutation, appears to be responsible for the multiple immune abnormalities of mev mice.
从1至2月龄存活的肌无力(mev)小鼠中获得了数百个杂交瘤。在检测的分泌免疫球蛋白的杂交瘤中,超过50%(17/33)对自身抗原有反应性,这支持了一个观点,即在mev小鼠中占主导的Ly-1 B细胞包含频繁的自身抗体形成细胞前体。这些自身抗体的某些特异性与已记录的mev小鼠病理生理学相关(抗胸腺细胞、抗红细胞、抗皮肤、抗肾脏和抗IgG);其他则针对在肌无力小鼠中以前未观察到但被认为与其他自身免疫性疾病有关的自身抗原(例如,内因子、转铁蛋白、髓磷脂碱性蛋白和甲状腺球蛋白)。大约三分之二(11/17)的自身反应性抗体对各种自身抗原有多特异性结合活性。对mev自身抗体中使用的V基因家族进行Northern印迹分析表明,VH家族的使用是随机的,而四个Vk基因家族的使用存在偏差。在检测的16种自身抗体中,12种使用了来自Vk1、4、10或19家族的Vk基因。这些Vk基因的使用模式与非自身免疫对照动物不同。总体而言,一种比VH或Vk基因座更普遍作用且由单一基因突变引起的免疫调节缺陷,似乎是mev小鼠多种免疫异常的原因。