Ziegler B, Strebelow M, Rjasanowski I, Schlosser M, Ziegler M
Institute of Pathophysiology, University Greifswald, Karlsburg, Germany.
Autoimmunity. 1998;28(2):61-8. doi: 10.3109/08916939809003868.
Autoantibodies to glutamic acid decarboxylase (GAD) are an important marker of the autoimmune-mediated beta-cell destruction in insulin-dependent (Type I) diabetes. However, these autoantibodies are also found in patients with Stiff-man syndrome (SMS) without onset of diabetes and some diabetic patients who initially present as non-insulin dependent (Type II) diabetes later becoming insulin-dependent, called as latent autoimmune diabetes in adults (LADA). To study the immune response to GAD in these LADA patients a competitive radiobinding assay based on murine monoclonal antibodies recognizing three different GAD regions was performed. The monoclonal antibodies against GAD recognize two different linear epitopes localized at the N- (amino acids 4-17) and C-terminus (amino acids 572-585) and one conformation-dependent epitope region (amino acids 221-442 IDDM-E1) known to be immunodominant for diabetes-associated autoantibodies. All LADA sera (20/20) reduced substantially the 125I-GAD binding of the monoclonal antibodies reactive with the conformation-dependent epitope region IDDM-E1 and only 20% of these sera additionally diminished the 125I-GAD65 binding by those monoclonals reactive with the both linear epitopes. The SMS sera completely abolished the GAD binding of all three monoclonals, reflecting a broader repertoire including an immune response against the IDDM-E1, a conformation-dependent GAD65 epitope region, also revealed if the SMS sera are diluted to equivalent antibody concentrations. In summary, our results show that diabetes-associated GAD autoantibodies even in adult patients with a late autoimmune process preferentially recognize a conformation-dependent middle GAD65 region. An immune response to all three GAD epitope regions is seldom in these LADA patients and only detectable in association with high antibody titres.
谷氨酸脱羧酶(GAD)自身抗体是胰岛素依赖型(I型)糖尿病中自身免疫介导的β细胞破坏的重要标志物。然而,在无糖尿病发作的僵人综合征(SMS)患者以及一些最初表现为非胰岛素依赖型(II型)糖尿病后来转变为胰岛素依赖型的糖尿病患者(称为成人隐匿性自身免疫糖尿病,LADA)中也发现了这些自身抗体。为了研究这些LADA患者对GAD的免疫反应,进行了一种基于识别三个不同GAD区域的鼠单克隆抗体的竞争性放射结合试验。抗GAD单克隆抗体识别位于N端(氨基酸4 - 17)和C端(氨基酸572 - 585)的两个不同线性表位以及一个已知对糖尿病相关自身抗体具有免疫显性的构象依赖性表位区域(氨基酸221 - 442,IDDM - E1)。所有LADA血清(20/20)均显著降低了与构象依赖性表位区域IDDM - E1反应的单克隆抗体的125I - GAD结合,并且这些血清中只有20%额外减少了与两个线性表位反应的单克隆抗体对125I - GAD65的结合。SMS血清完全消除了所有三种单克隆抗体的GAD结合,反映出更广泛的免疫反应谱,包括针对IDDM - E1(一种构象依赖性GAD65表位区域)的免疫反应,当SMS血清稀释至等效抗体浓度时也可显示出这一点。总之,我们的结果表明,即使在自身免疫过程较晚的成年患者中,与糖尿病相关的GAD自身抗体也优先识别构象依赖性的中间GAD65区域。在这些LADA患者中,对所有三个GAD表位区域的免疫反应很少见,仅在高抗体滴度时可检测到。