Lindstrom J M, Lennon V A, Seybold M E, Whittingham S
Ann N Y Acad Sci. 1976;274:254-74. doi: 10.1111/j.1749-6632.1976.tb47691.x.
Stucture of acetylcholine receptor protein (AChR) purified from Electrophorus electricus (eel) by affinity chromatography is described. AChR is detected in extracts from human muscle, rat muscle, and rat thymus. Rats immunized with eel AChR develop humoral antibodies, a small fraction of which recognize AChR from rat muscle. Rats immunized with AChR exhibit myasthenia, but those immunized with denatured AChR do not. Immunoglobulin fraction of antisera to eel AChR can block the activity of AChR in electroplaques. Sera from patients with myasthenia gravis contain antibodies to AChR from human muscle detectabe at an average value 300-fold the background level in sera from nonmyasthenics. Relationship of thymoma and disease intensity to antibody titer is examined. The chronic phase of EAMG appears a good model of MG, since in both cases similar concentrations of 7-S immunoglobulin against determinants on muscle AChR other than the toxin binding site are found. Assay of anti-AChR antibody in sera from MG patients using AChR from rat muscle gives titers 10%-15% of those obtained using AChR from human muscle, and using AChR from eel gives negligible titers. The assay method described for assaying antibodies against AChR from human muscle is suggested as a diagnostic test for MG.
描述了通过亲和层析从电鳗（Electrophorus electricus）中纯化得到的乙酰胆碱受体蛋白（AChR）的结构。在人肌肉、大鼠肌肉和大鼠胸腺的提取物中检测到了AChR。用鳗AChR免疫的大鼠产生体液抗体，其中一小部分能识别大鼠肌肉中的AChR。用AChR免疫的大鼠出现肌无力，但用变性AChR免疫的大鼠则不会。鳗AChR抗血清的免疫球蛋白部分可阻断电板中AChR的活性。重症肌无力患者的血清中含有针对人肌肉AChR的抗体，其平均水平比非肌无力患者血清中的背景水平高300倍。研究了胸腺瘤和疾病严重程度与抗体滴度的关系。实验性自身免疫性重症肌无力（EAMG）的慢性期似乎是重症肌无力（MG）的一个良好模型，因为在这两种情况下，都发现了针对肌肉AChR上除毒素结合位点以外的决定簇的7-S免疫球蛋白浓度相似。用大鼠肌肉AChR检测重症肌无力患者血清中的抗AChR抗体，其滴度为用人肌肉AChR检测时的10%-15%，而用鳗AChR检测时滴度可忽略不计。建议将所描述的检测针对人肌肉AChR抗体的检测方法作为重症肌无力的诊断试验。
