Mygland A, Tysnes O B, Aarli J A, Matre R, Gilhus N E
Department of Neurology, University of Bergen, Norway.
J Autoimmun. 1993 Aug;6(4):507-15. doi: 10.1006/jaut.1993.1042.
Patients with myasthenia gravis (MG) and thymoma often have autoantibodies to the ryanodine receptor (RyR), a ligand-gated Ca(2+)-release channel in the sarcoplasmic reticulum of striated muscle. Using an ELISA, we studied the Ig isotype and IgG subclass distribution of RyR autoantibodies in sera from 30 MG patients with thymoma. RyR IgG antibodies were detected in 16/30 sera. 14/30 sera had IgG1 RyR antibodies, and 15/30 sera had IgG3 RyR antibodies. All sera with IgG1 RyR antibodies also contained IgG3 antibodies. A small number of sera had low amounts of IgG2, IgG4, IgA or IgM RyR antibodies. The RyR autoantibodies are therefore mainly expressed in the IgG1 and IgG3 subclasses. The RyR autoantibodies may be of pathogenic significance by interfering with the mechanism of excitation-contraction coupling in striated muscle.
重症肌无力(MG)合并胸腺瘤的患者常常产生针对兰尼碱受体(RyR)的自身抗体,RyR是横纹肌肌浆网中的一种配体门控性钙释放通道。我们采用酶联免疫吸附测定法(ELISA),研究了30例MG合并胸腺瘤患者血清中RyR自身抗体的免疫球蛋白(Ig)同种型和IgG亚类分布情况。在30份血清中,有16份检测到RyR IgG抗体。30份血清中有14份含有IgG1 RyR抗体,15份含有IgG3 RyR抗体。所有含有IgG1 RyR抗体的血清也都含有IgG3抗体。少数血清含有少量的IgG2、IgG4、IgA或IgM RyR抗体。因此,RyR自身抗体主要在IgG1和IgG3亚类中表达。RyR自身抗体可能通过干扰横纹肌兴奋 - 收缩偶联机制而具有致病意义。