Autoantibodies to Low-Density Lipoprotein Receptor-Related Protein 4 in Myasthenia Gravis
Autoantibodies to Low-Density Lipoprotein Receptor-Related Protein 4 in Myasthenia Gravis
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DOI：
10.1002/ana.22312
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发表时间：
2011-02-01
影响因子：
11.2
通讯作者：
Yamanashi, Yuji
中科院分区：
文献类型：
--
作者：
Higuchi, Osamu;Hamuro, Johko;Yamanashi, Yuji
重症肌无力 (MG) 是一种神经肌肉接头自身免疫性疾病，其中乙酰胆碱受体 (AChR)、肌肉特异性激酶 (MuSK) 和低密度脂蛋白 (LDL) 受体相关蛋白 4 (Lrp4) 至关重要。大约 80% 和 0% 至 10% 的全身性 MG 患者分别具有针对 AChR 和 MuSK 的自身抗体，但其他患者的致病因素则难以捉摸。在这里，我们发现一部分 AChR 抗体阴性患者具有 Lrp4 自身抗体。这些抗体抑制 Lrp4 与其配体的结合，主要属于免疫球蛋白 G1 (IgG1) 亚类（一种补体激活剂）。这些发现共同表明 Lrp4 抗体参与了 AChR 抗体阴性 MG 的发病机制。安神经学 2011；69:418-422
Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction, where acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein (LDL) receptor-related protein 4 (Lrp4) are essential. About 80% and 0% to 10% of patients with generalized MG have autoantibodies to AChR and MuSK, respectively, but pathogenic factors are elusive in others. Here we show that a proportion of AChR antibody-negative patients have autoantibodies to Lrp4. These antibodies inhibit binding of Lrp4 to its ligand and predominantly belong to the immunoglobulin G1 (IgG1) subclass, a complement activator. These findings together indicate the involvement of Lrp4 antibodies in the pathogenesis of AChR antibody-negative MG. ANN NEUROL 2011;69:418-422