Ocular and generalized myasthenia gravis induced by human acetylcholine receptor ? subunit immunization

被引：14
|
作者：
Wu, Xiaorong [2 ]
Tuzun, Erdem [1 ]
Li, Jing [1 ]
Xiao, Tianlin [1 ]
Saini, Shamsher S. [1 ]
Qi, Huibin [1 ]
Allman, Windy [1 ]
Christadoss, Premkumar [1 ]
机构：
[1] Univ Texas Med Branch, Dept Microbiol & Immunol, Galveston, TX 77555 USA
[2] Cent S Univ, Xiangya Hosp 2, Dept Ophthalmol, Changsha, Hunan, Peoples R China
来源：
MUSCLE & NERVE | 2012年 / 45卷 / 02期
关键词：
acetylcholine receptor ? subunit; autoimmune disease; extraocular muscles; myasthenia gravis; ocular myasthenia gravis; HLA TRANSGENIC MICE; EXTRAOCULAR-MUSCLE; GENE-EXPRESSION; GAMMA-SUBUNIT; SKELETAL-MUSCLE; ALPHA-SUBUNIT; MOUSE MODEL; PATHOGENESIS; INDUCTION; MECHANISM;
D O I：
10.1002/mus.22273
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
Introduction: HLA-DQ8 transgenic mice develop ocular myasthenia gravis (oMG), which then progresses to generalized MG (gMG) when immunized with the human acetylcholine receptor (H-AChR) a subunit. Because the fetal AChR ? subunit is expressed in adult extraocular muscles, we anticipated that ? subunit immunization would generate an immune response to mouse AChR and induce MG in mice. Results: H-AChR ? subunit immunization in HLA-DQ8 mice induced an autoimmune response to mouse AChR and led to the destruction of AChR in the neuromuscular junction (NMJ) by anti-AChR antibody and complement activation, and it triggered upregulation of AChR gene transcription. Conclusion: Our findings indicate that oMG may be induced by immunity to the AChR ? subunit. Muscle Nerve, 2012
引用
收藏
页码：209 / 216
页数：8
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