乙酰胆碱受体抗体阴性重症肌无力患者血清对细胞培养中乙酰胆碱受体和肌肉特异性激酶的影响。

Effect of sera from AChR-antibody negative myasthenia gravis patients on AChR and MuSK in cell cultures.

作者信息

Farrugia Maria Elena, Bonifati Domenico Marco, Clover Linda, Cossins Judy, Beeson David, Vincent Angela

机构信息

Neurosciences Group, Weatherall Institute of Molecular Medicine, Department of Clinical Neurology, University of Oxford, UK.

出版信息

J Neuroimmunol. 2007 Apr;185(1-2):136-44. doi: 10.1016/j.jneuroim.2007.01.010. Epub 2007 Mar 1.

Abstract

A proportion of patients with myasthenia gravis (MG) do not have antibodies to the acetylcholine receptor (AChR). Some of these patients have antibodies to muscle specific kinase (MuSK), whereas others have neither antibody (seronegative MG, SNMG). Both MuSK antibody positive MG (MuSK-MG) and SNMG are antibody-mediated diseases but how they cause neuromuscular junction failure is not clear. One possibility is that they reduce the clustering and expression of AChRs. We looked at the effects of MuSK-MG and SNMG sera/IgG on surface AChR distribution and expression, and AChR subunit and MuSK mRNA by quantitative RT-PCR, in TE671 and C2C12 myotubes. In TE671 cells MuSK-MG sera reduced AChR expression by about 20%, but had no effect on AChR subunit or MuSK mRNA expression. In C2C12 myotubes, MuSK-MG sera caused a reduction in the number of agrin-induced clusters, but the clusters became larger and there was no significant effect on total surface AChR numbers or AChR subunit or MuSK mRNA. By contrast, SNMG sera not only reduced AChR numbers by about 20% in TE671 cells, but modestly upregulated AChR gamma subunit expression in TE671 cells and both AChR gamma subunit and MuSK expression in C2C12 myotubes. Thus, although these results have, disappointingly, demonstrated little effect of MuSK antibodies on AChR expression, they do imply that SNMG antibodies act on AChR-associated pathways.

摘要

一部分重症肌无力(MG)患者没有抗乙酰胆碱受体(AChR)抗体。其中一些患者有抗肌肉特异性激酶(MuSK)抗体,而另一些患者则两种抗体都没有(血清阴性MG,SNMG)。MuSK抗体阳性MG(MuSK-MG)和SNMG都是抗体介导的疾病,但它们如何导致神经肌肉接头功能障碍尚不清楚。一种可能性是它们会减少AChR的聚集和表达。我们研究了MuSK-MG和SNMG血清/IgG对TE671和C2C12肌管表面AChR分布和表达、AChR亚基以及MuSK mRNA的影响,采用定量逆转录聚合酶链反应(RT-PCR)进行检测。在TE671细胞中,MuSK-MG血清使AChR表达降低约20%,但对AChR亚基或MuSK mRNA表达没有影响。在C2C12肌管中,MuSK-MG血清导致聚集蛋白诱导的簇数量减少,但簇变得更大,对总表面AChR数量、AChR亚基或MuSK mRNA没有显著影响。相比之下,SNMG血清不仅使TE671细胞中的AChR数量减少约20%,而且适度上调了TE671细胞中AChRγ亚基的表达以及C2C12肌管中AChRγ亚基和MuSK的表达。因此,尽管令人失望的是,这些结果表明MuSK抗体对AChR表达几乎没有影响,但它们确实意味着SNMG抗体作用于与AChR相关的途径。

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