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首页> 外文期刊>Neuron >Autoimmunity to munc-18 in Rasmussen's encephalitis.
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Autoimmunity to munc-18 in Rasmussen's encephalitis.

机译:拉斯穆森脑炎对mun​​c-18的自身免疫。

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摘要

Rasmussen's encephalitis (RE) is a rare disease of the central nervous system characterized by severe epileptic seizures, progressive degeneration of a single cerebral hemisphere, and autoimmunity directed against glutamate receptor subunit, GluR3. We report here the identification of high-titer autoantibodies directed against munc-18 in the serum of a single patient with RE previously shown to have anti-GluR3 antibodies. Munc-18 is an intracellular protein residing in presynaptic terminals, which is required for secretion of neurotransmitters. These findings are consistent with the possibility of intermolecular epitope spreading between GluR3, a postsynaptic cell surface protein, and munc-18, a presynaptic intracellular protein. Immune attack on these two proteins, which participate at distinct steps of synaptic transmission, could act in an additive or synergistic manner to impair synaptic function and lead to seizures and neuronal death.
机译:拉斯穆森脑炎(RE)是中枢神经系统的一种罕见疾病,其特征是严重的癫痫发作,单个脑半球进行性变性以及针对谷氨酸受体亚基GluR3的自身免疫。我们在这里报告了针对先前被证明具有抗GluR3抗体的RE患者的血清中针对munc-18的高滴度自身抗体的鉴定。 Munc-18是一种位于突触前末端的细胞内蛋白,是神经递质分泌所必需的。这些发现与分子间表位在突触后细胞表面蛋白GluR3和突触前细胞内蛋白munc-18之间扩散的可能性一致。对这两种蛋白质的免疫攻击会参与突触传递的不同步骤,可能以累加或协同方式起作用,损害突触功能并导致癫痫发作和神经元死亡。

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