一项利用TCR库的CDR3谱型分析对儿童期重症肌无力发病诱导因素的研究。

A study of the factors inducing the development of childhood-onset myasthenia gravis using CDR3 spectratyping analysis of the TCR repertoire.

作者信息

Dokai Hidenori, Nomura Yoshiko, Fujikawa Yoshinao, Nihei Koichi, Segawa Masaya, Shinomiya Noriaki

机构信息

Toho University Medical Center, Ohashi Hospital, 2nd Department of Pediatrics, 2-17-6 Ohashi Meguro-ku, Tokyo 153-8515, Japan.

出版信息

J Neuroimmunol. 2007 Jul;187(1-2):192-200. doi: 10.1016/j.jneuroim.2007.04.021. Epub 2007 Jun 8.

DOI:10.1016/j.jneuroim.2007.04.021

PMID:17560663

Abstract

Myasthenia gravis (MG) is an autoimmune disease. AChR-specific autologous helper T (Th) cells are essential to the pathogenesis of MG. Factors correlated with the development of childhood-onset MG are unknown. In longitudinal studies, we found TCR Vbeta 2/5.1/6/7 usage in the development or relapse phases, but not in the remission phase. We also found that TCR Vbeta 8/9/13.1/15/18/20 usage persisted. The polyclonally expanded TCR Vbeta 2/5.1/6/7 by CDR3 spectratyping was found to be associated with the development of disease. These data suggest that in patients with childhood-onset MG, stimuli such as superantigens induced by a preceding infection, which cause development of the polyclonal pattern in TCR Vbeta families, play an important role in the development of the disease.

摘要

重症肌无力（MG）是一种自身免疫性疾病。乙酰胆碱受体特异性自体辅助性T（Th）细胞对MG的发病机制至关重要。与儿童期发病MG发展相关的因素尚不清楚。在纵向研究中，我们发现在疾病发展或复发阶段存在TCR Vβ 2/5.1/6/7的使用情况，但在缓解阶段则没有。我们还发现TCR Vβ 8/9/13.1/15/18/20的使用情况持续存在。通过CDR3谱型分析发现，多克隆扩增的TCR Vβ 2/5.1/6/7与疾病的发展相关。这些数据表明，在儿童期发病的MG患者中，先前感染诱导的超抗原等刺激因素导致TCR Vβ家族出现多克隆模式，在疾病发展中起重要作用。