IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel

IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel
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DOI:
10.1084/jem.20050304
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发表时间:
2005-08-15
影响因子:
15.3
通讯作者:
Hinson, SR
Hinson, SR
中科院分区:
医学1区
文献类型:
--
作者:
Lennon, VA;Kryzer, TJ;Hinson, SR

文献摘要

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视神经脊髓炎(NMO)是一种选择性影响视神经和脊髓的炎性脱髓鞘疾病。它被认为是多发性硬化症(MS)的一种严重变体,经常被误诊为MS,但预后和最佳治疗方法不同。血清免疫球蛋白G自身抗体(NMO- igg)作为NMO的特异性标志物。在这里,我们发现NMO-IgG选择性地与水通道蛋白4结合,水通道蛋白4是位于血脑屏障的星形细胞足突中的糖醛酸蛋白复合物的一个组成部分。NMO可能是一类新型自身免疫性通道病的第一例。
Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that selectively affects optic nerves and spinal cord. It is considered a severe variant of multiple sclerosis (MS), and frequently is misdiagnosed as MS, but prognosis and optimal treatments differ. A serum immunoglobulin G autoantibody (NMO-IgG) serves as a specific marker for NMO. Here we show that NMO-IgG binds selectively to the aquaporin-4 water channel, a component of the dystroglycan protein complex located in astrocytic foot processes at the blood-brain barrier. NMO may represent the first example of a novel class of autoimmune channelopathy.