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重症肌无力中对兰尼碱受体和肌联蛋白的自身免疫与GM同种异型相关。

Autoimmunity to ryanodine receptor and titin in myasthenia gravis is associated with GM allotypes.

作者信息

Skeie G O, Pandey J P, Aarli J A, Gilhus N E

机构信息

Department of Neurology, University of Bergen, Norway.

出版信息

Autoimmunity. 1997;26(2):111-6. doi: 10.3109/08916939709003854.

DOI:10.3109/08916939709003854
PMID:9546812
Abstract

Myasthenia gravis (MG) is mediated by autoantibodies against the acetylcholine receptor at the muscle endplate. Some MG patients have in addition antibodies (Ab) to the skeletal muscle proteins ryanodine receptor (RyR) and titin. We have examined GM and KM allotypes, RyR and titin Ab in 44 MG patients (37 thymoma patients and 7 non-thymoma, late-onset patients) and 292 non-MG controls to see if GM/KM allotypes associate with differences in autoantibody production. All patients had titin Ab, and 15 thymoma patients had also RyR Ab. The phenotype GM 1, 2, 3 23 5, 21 was significantly increased in the patients with titin Ab compared with the non-MG controls (chi2 = 4.93, p < 0.05). Thymoma patients with RyR Ab had a higher frequency of the GM 3 23 5 phenotype compared with RyR Ab negative patients and controls (chi2 = 7.1, p < 0.05). KM allotypes did not differ between RyR Ab positive or titin Ab positive patients and controls. GM phenotypes may thus be associated with an autoimmune response against the muscle proteins titin and RyR in MG patients.

摘要

重症肌无力(MG)由针对肌肉终板乙酰胆碱受体的自身抗体介导。一些MG患者还存在针对骨骼肌蛋白兰尼碱受体(RyR)和肌联蛋白的抗体(Ab)。我们检测了44例MG患者(37例胸腺瘤患者和7例非胸腺瘤、晚发型患者)和292例非MG对照的GM和KM同种异型、RyR和肌联蛋白抗体,以观察GM/KM同种异型是否与自身抗体产生的差异相关。所有患者均有肌联蛋白抗体,15例胸腺瘤患者也有RyR抗体。与非MG对照相比,有肌联蛋白抗体的患者中GM 1、2、3 23 5、21表型显著增加(χ2 = 4.93,p < 0.05)。与RyR抗体阴性患者及对照相比,有RyR抗体的胸腺瘤患者GM 3 23 5表型频率更高(χ2 = 7.1,p < 0.05)。RyR抗体阳性或肌联蛋白抗体阳性患者与对照之间的KM同种异型无差异。因此,GM表型可能与MG患者针对肌肉蛋白肌联蛋白和RyR的自身免疫反应相关。

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