Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome
Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome
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DOI：
10.1002/ana.20539
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发表时间：
2005-08-01
影响因子：
11.2
通讯作者：
Preissner, KT
中科院分区：
文献类型：
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作者：
Blaes, F;F端hlhuber, V;Preissner, KT
儿童视阵挛-肌阵挛综合征可合并或不合并神经母细胞瘤。对于这两种形式，自身免疫的发病机制都进行了讨论。我们在这里表明，大多数患有眼阵挛-肌阵挛综合征的儿童(10/14)具有与分离的大鼠小脑颗粒神经元表面结合的自身抗体。在一些患者中，这些抗体被与无处不在的表面抗原结合的免疫球蛋白所掩盖，这些抗原可以通过与非神经元控制细胞系HEK 293预先孵育来去除。一项新引入的竞争结合分析表明，表面结合是针对不同患者的同一自身抗原。因此，我们假设眼阵挛-肌阵挛综合征可能是针对神经元表面蛋白的自身免疫过程的结果。
Childhood opsoclonus-myoclonus syndrome can occur with or without associated neuroblastoma. An autoimmune pathogenesis has been discussed for both forms. We show here that the majority of children with opsoclonus-myoclonus syndrome (10/14) have autoantibodies binding to the surface of isolated rat cerebellar granular neurons. In some patients, these antibodies are masked by IgG binding to ubiquitous surface antigens, which could be removed by preincubation with the nonneuronal control cell line HEK 293. A newly introduced competitive binding assay showed that the surface binding is directed against the same autoantigen in different patients. Therefore, we hypothesize that opsoclonus-myoclonus syndrome may be the result of an autoimmune process against a neuronal surface protein.