重症肌无力胸腺中肌联蛋白主要免疫原性区域的胸腺表达

Thymic expression of the main immunogenic region of titin in thymomatous myasthenia gravis.

作者信息

He Z F, Lv W, Qiao J, Chen Z M, Pang L W, Chen X J

机构信息

Department of Cardiothoracic Surgery, Sir Run Run Shaw Hospital, Zhejiang University, Hangzhou, China.

出版信息

J Int Med Res. 2010 Jul-Aug;38(4):1324-32. doi: 10.1177/147323001003800414.

DOI:10.1177/147323001003800414

PMID:20926005

Abstract

Antititin antibody occurs in the serum of myasthenia gravis (MG) patients with thymoma (MGT), and is a diagnostic marker for the disease. The mechanism that triggers MGT development is, however, unclear. This study evaluated the role of the main immunogenic region (MIR) of titin in MGT pathogenesis. Titin MIR antiserum (antibody titre 1:16) was obtained and an in situ immunohistochemical study of thymomatous tissue samples was performed. Strong immunostaining for titin MIR was observed on epithelial cell membranes in MGT patients, and the degree of immunostaining was directly proportional to the number of epithelial cells in thymomatous tissue. Serum antititin antibody levels were closely related to titin MIR levels in thymoma cells; however, titin MIR levels did not appear to be related to MG severity. Antititin antibody may be a good surrogate marker for thymoma but is probably not involved in the pathogenesis of MGT.

摘要

抗肌联蛋白抗体出现在患有胸腺瘤的重症肌无力（MG）患者血清中，是该疾病的一种诊断标志物。然而，引发胸腺瘤性重症肌无力（MGT）发展的机制尚不清楚。本研究评估了肌联蛋白主要免疫原性区域（MIR）在MGT发病机制中的作用。获取了肌联蛋白MIR抗血清（抗体效价1:16），并对胸腺瘤组织样本进行了原位免疫组化研究。在MGT患者的上皮细胞膜上观察到了针对肌联蛋白MIR的强免疫染色，且免疫染色程度与胸腺瘤组织中的上皮细胞数量成正比。血清抗肌联蛋白抗体水平与胸腺瘤细胞中的肌联蛋白MIR水平密切相关；然而，肌联蛋白MIR水平似乎与MG严重程度无关。抗肌联蛋白抗体可能是胸腺瘤的一个良好替代标志物，但可能不参与MGT的发病机制。