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[静脉注射免疫球蛋白对实验性重症肌无力的调节作用]

[Modulation of experimental myasthenia gravis by IVIg].

作者信息

Berrih-Aknin S, Aissaoui A, Yamamoto M, Kaveri S V

机构信息

CNRS ESA 8078, Université Paris Sud, Hôpital Marie Lannelongue, Plessis Robinson.

出版信息

Ann Med Interne (Paris). 2000 May;151 Suppl 1:1S25-9.

Abstract

Myasthenia Gravis (MG) is an autoimmune disease mediated by antibodies directed against the acetylcholine receptor (AChR). Treatment by IVIg is effective in acute forms of myasthenia gravis. In order to determine the in vivo effects of the various fractions of human immunoglobulins, we used an experimental model of myasthenia gravis in SCID mice. To this end, thymic cells from MG patients are transferred to these mice according to a well defined protocol. When establishing of the model, we noticed the appearance of anti-AChR antibodies and the loss of AChR expression at the muscle level. After treatment with IVIgG or IVIgM, the mice displayed a lower anti-AChR antibody titer compared to control mice (albumin treated) and the loss of the AChR number at the muscle was significantly reduced. These results obtained from one MG patient indicate that the human immunoglobulin preparations induce significant effects on pathogenic parameters in the SCID mouse model. Therefore this model is interesting to approach the mechanisms of action of human immunoglobulins and deserves further investigation.

摘要

重症肌无力(MG)是一种由针对乙酰胆碱受体(AChR)的抗体介导的自身免疫性疾病。静脉注射免疫球蛋白(IVIg)治疗对重症肌无力的急性形式有效。为了确定人免疫球蛋白各组分的体内作用,我们在重症联合免疫缺陷(SCID)小鼠中使用了重症肌无力的实验模型。为此,根据明确的方案将重症肌无力患者的胸腺细胞转移到这些小鼠体内。在建立模型时,我们注意到抗AChR抗体的出现以及肌肉水平上AChR表达的丧失。用IVIgG或IVIgM治疗后,与对照小鼠(白蛋白治疗)相比,小鼠的抗AChR抗体滴度较低,并且肌肉中AChR数量的丧失显著减少。从一名重症肌无力患者获得的这些结果表明,人免疫球蛋白制剂对SCID小鼠模型中的致病参数有显著影响。因此,该模型对于研究人免疫球蛋白的作用机制很有意义,值得进一步研究。

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