Tremble J, Morgenthaler N G, Vlug A, Powers A C, Christie M R, Scherbaum W A, Banga J P
Department of Medicine, King's College School of Medicine, London, United Kingdom.
J Clin Endocrinol Metab. 1997 Aug;82(8):2664-70. doi: 10.1210/jcem.82.8.4171.
Antibodies to glutamic acid decarboxylase-65 (GAD65) are present in a number of autoimmune disorders, such as insulin-dependent (type 1) diabetes mellitus (IDDM), stiff man syndrome, and polyendocrine autoimmune disease. Antibodies to GAD in IDDM patients usually recognize conformation-dependent regions on GAD65 and rarely bind to the second isoform, glutamic acid decarboxylase-67 (GAD67). In contrast, those present in stiff man syndrome and polyendocrine disease commonly target the second isoform (GAD67) and include antibodies that are less dependent on the conformation of the molecule. By immortalizing peripheral blood B cells with Epstein-Barr virus, we have generated three human IgG autoantibodies, termed b35, b78, and b96, to GAD65 from one patient with multiple autoantibodies to endocrine organs and Graves' disease. All three autoantibodies are of the IgG1 isotype, with islet cell activity, and do not react with GAD67. The regions on GAD65 recognized by the three autoantibodies have been investigated by immunoprecipitation with a series of chimeras, by binding to denatured and reduced antigens, and using protein footprinting techniques. Using chimeric GAD proteins, we have shown that b35 targets the IDDM-E1 region of GAD65 (amino acids 240-435) whereas both b78 and b96 target the IDDM-E2 region of GAD65 (amino acids 451-570). Furthermore, examination of binding to recombinant GAD65 and GAD67 by Western blotting revealed some differences in epitope recognition, where only b78 bound denatured and reduced GAD65. However, b35, b78, and b96 autoantibodies had different footprinting patterns after trypsin treatment of immune complexes with GAD65, again indicating different epitope recognition. Our results indicate that antibodies to GAD65 present in nondiabetic patients with multiple autoantibodies to endocrine organs show similarities to those in IDDM (by targeting IDDM-E1 and IDDM-E2 regions of GAD65) as well as subtle differences in epitope recognition (such as binding to denatured and reduced GAD65 and by protein footprinting). Thus, the GAD65 epitopes recognized by autoantibodies in different autoimmune diseases may overlap and be more heterogeneous than previously recognized.
谷氨酸脱羧酶65(GAD65)抗体存在于多种自身免疫性疾病中,如胰岛素依赖型(1型)糖尿病(IDDM)、僵人综合征和多内分泌自身免疫性疾病。IDDM患者体内的GAD抗体通常识别GAD65上依赖构象的区域,很少与第二种同工型谷氨酸脱羧酶67(GAD67)结合。相比之下,僵人综合征和多内分泌疾病患者体内的抗体通常靶向第二种同工型(GAD67),包括一些对分子构象依赖性较小的抗体。通过用爱泼斯坦-巴尔病毒使外周血B细胞永生化,我们从一名患有多种内分泌器官自身抗体和格雷夫斯病的患者体内产生了三种针对GAD65的人IgG自身抗体,分别称为b35、b78和b96。这三种自身抗体均为IgG1同种型,具有胰岛细胞活性,且不与GAD67反应。通过用一系列嵌合体进行免疫沉淀、与变性和还原抗原结合以及使用蛋白质足迹技术,研究了这三种自身抗体识别的GAD65区域。利用嵌合GAD蛋白,我们发现b35靶向GAD65的IDDM-E1区域(氨基酸240 - 435),而b78和b96均靶向GAD65的IDDM-E2区域(氨基酸451 - 570)。此外,通过蛋白质印迹法检测与重组GAD65和GAD67的结合情况,发现表位识别存在一些差异,其中只有b78能与变性和还原的GAD65结合。然而,在用胰蛋白酶处理与GAD65形成的免疫复合物后,b35、b78和b96自身抗体具有不同的足迹模式,再次表明表位识别不同。我们的结果表明,患有多种内分泌器官自身抗体的非糖尿病患者体内的GAD65抗体与IDDM患者体内的抗体有相似之处(通过靶向GAD65的IDDM-E1和IDDM-E2区域),但在表位识别上也存在细微差异(如与变性和还原的GAD65结合以及蛋白质足迹分析)。因此,不同自身免疫性疾病中自身抗体识别的GAD65表位可能重叠,且比之前认为的更加异质化。