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重症肌无力患者血清对人成肌细胞培养的影响。

Effects of myasthenia gravis patient sera on human myoblast cultures.

作者信息

Luckman S P, Skeie G O, Helgeland G, Gilhus N E

机构信息

Institute of Clinical Medicine, Section for Neurology, University of Bergen and Haukeland University Hospital, Bergen, Norway.

出版信息

Acta Neurol Scand Suppl. 2006;183:28-32. doi: 10.1111/j.1600-0404.2006.00611.x.

Abstract

OBJECTIVES

To investigate the role of acetylcholine receptor (AChR) and other anti-muscle autoantibodies in myasthenia gravis (MG). Since many of these autoantibodies target proteins with structural or signalling functions, we examined the effect of MG sera on muscle cell morphology.

MATERIALS AND METHODS

Primary human myoblast cultures were exposed to MG sera and morphological changes observed by light and fluorescence microscopy.

RESULTS

MG patient sera caused changes in cell shape (cell retraction) and led to the formation of inclusion bodies and intracellular vesicles. A disordered arrangement of actin microfilaments was also observed. The effects were not complement-mediated, were both dose- and time-dependent, and appeared to correlate with disease severity of the MG donor.

CONCLUSION

The factors responsible for these effects in vitro may also play a role in the pathogenesis of MG in vivo. Further study of these factors may improve our understanding of MG pathogenesis.

摘要

目的

研究乙酰胆碱受体(AChR)及其他抗肌肉自身抗体在重症肌无力(MG)中的作用。由于这些自身抗体中有许多靶向具有结构或信号功能的蛋白质,我们检测了MG血清对肌肉细胞形态的影响。

材料与方法

将原代人成肌细胞培养物暴露于MG血清中,通过光学显微镜和荧光显微镜观察形态变化。

结果

MG患者血清导致细胞形状改变(细胞收缩),并导致包涵体和细胞内小泡的形成。还观察到肌动蛋白微丝排列紊乱。这些效应并非补体介导,具有剂量和时间依赖性,且似乎与MG供体的疾病严重程度相关。

结论

在体外造成这些效应的因素可能在MG的体内发病机制中也起作用。对这些因素的进一步研究可能会增进我们对MG发病机制的理解。

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