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单克隆抗乙酰胆碱受体抗体作为重症肌无力患者人类乙酰胆碱受体的探针。

Monoclonal anti-acetylcholine receptor antibodies as probes for human acetylcholine-receptor in myasthenia gravis.

作者信息

Vincent A, Whiting P J, Heidenreich F, Roberts A

机构信息

Department of Neurological Sciences, Royal Free Hospital School of Medicine, London.

出版信息

J Recept Res. 1988;8(1-4):143-59. doi: 10.3109/10799898809048984.

Abstract

Monoclonal antibodies have been shown to bind to five regions on human acetylcholine receptor, each probably consisting of a discrete epitope on the extracellular surface. Two of these regions are equivalent to the 'main immunogenic region', and the other three appear to be close to the a-Bungarotoxin binding sites. These antibodies have been used to probe differences in myasthenia gravis anti-acetylcholine receptor antibodies, to locate acetylcholine receptor in thymic tissue, and to look for naturally-occurring anti-idiotype antibodies. Anti-acetylcholine receptor antibody specificities differ between groups of patients defined by their age of onset, thymic pathology and HLA associations. Anti-AChR synthesised by the thymus in young onset patients has similar specificity to that found in the individual's serum, and may be stimulated by the presence of AChR on thymic myoid cells. However, myoid cells (defined by staining with anti-troponin and anti-myosin antibodies) do not appear to differ between control and myasthenia gravis patients and show no obvious involvement in an immunological reaction. There was no convincing evidence for the presence of anti-idiotype antibodies in myasthenia gravis patients.

摘要

单克隆抗体已被证明可与人乙酰胆碱受体上的五个区域结合,每个区域可能由细胞外表面上的一个离散表位组成。其中两个区域等同于“主要免疫原性区域”,另外三个区域似乎靠近α-银环蛇毒素结合位点。这些抗体已被用于探究重症肌无力抗乙酰胆碱受体抗体的差异,在胸腺组织中定位乙酰胆碱受体,并寻找天然存在的抗独特型抗体。抗乙酰胆碱受体抗体的特异性在根据发病年龄、胸腺病理学和HLA关联定义的患者组之间存在差异。年轻发病患者胸腺合成的抗AChR与个体血清中发现的抗AChR具有相似的特异性,并且可能受到胸腺肌样细胞上AChR的存在的刺激。然而,肌样细胞(通过用抗肌钙蛋白和抗肌球蛋白抗体染色来定义)在对照患者和重症肌无力患者之间似乎没有差异,并且在免疫反应中没有明显参与。没有令人信服的证据表明重症肌无力患者中存在抗独特型抗体。

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