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肌球蛋白是巨细胞病毒相关性格林-巴利综合征的一个可能的靶标分子。

Moesin is a possible target molecule for cytomegalovirus-related Guillain-Barré syndrome.

机构信息

From the Departments of Molecular Diagnosis (S.S., M.S., K. Sogawa, T.K., M.I., M.B., T.I., K.N., K. Sato, K.M., F.N.) and Neurology (M.M., S.M., M.B., K. Shibuya, Y.S., S.K.), Graduate School of Medicine, Chiba University; and Department of Physics (Y.K.), School of Science, Kitasato University, Kanagawa, Japan.

出版信息

Neurology. 2014 Jul 8;83(2):113-7. doi: 10.1212/WNL.0000000000000566. Epub 2014 Jun 11.

Abstract

OBJECTIVE

Previous histochemical studies in the demyelinating form of Guillain-Barré syndrome (GBS), acute inflammatory demyelinating polyneuropathy (AIDP), have shown complement deposition on the surface of Schwann cells, and therefore unknown epitopes would be present on the outer surface of Schwann cells.

METHODS

We used a proteomic-based approach to search for the target molecules of AIDP in the extracted proteins from schwannoma cells. Sera were obtained from 40 patients with GBS, 31 controls with inflammatory disease, and 46 normal controls.

RESULTS

We found that patients with AIDP after cytomegalovirus (CMV) infection have serum autoantibodies against membrane-organizing extension spike protein (moesin), which is expressed in the Schwann cell processes at the nodes of Ranvier and is crucial for myelination. Of the 40 patients with GBS, 6 had recent CMV infection and 5 of them (83%) had high levels of serum immunoglobulin G antibodies against moesin. The anti-moesin antibodies were found in none of the control subjects with disease including 5 with CMV infection but no neuropathy, and only 2 (4%) of the 46 normal control subjects. Immunocytochemistry showed that moesin was stained at the distal tips of schwannoma cells by sera from the patients with CMV-related AIDP but not by sera from controls.

CONCLUSION

Moesin is a possible immunologic target molecule of pathogenic autoantibodies in patients with CMV-related AIDP.

CLASSIFICATION OF EVIDENCE

This study provides Class II evidence that levels of serum anti-moesin antibodies accurately distinguishes CMV-related AIDP from non-CMV-related AIDP (sensitivity 83%, specificity 93%).

摘要

目的

先前在吉兰-巴雷综合征(GBS)脱髓鞘型和急性炎症性脱髓鞘性多发性神经病(AIDP)的组织化学研究中表明,补体沉积在施万细胞表面,因此,施万细胞外表面存在未知抗原表位。

方法

我们使用基于蛋白质组学的方法,从施万细胞瘤中提取的蛋白质中寻找 AIDP 的靶分子。采集了 40 例 GBS 患者、31 例炎症性疾病对照者和 46 名正常对照者的血清。

结果

我们发现,巨细胞病毒(CMV)感染后发生 AIDP 的患者,血清中存在针对膜组织延伸刺突蛋白(moesin)的自身抗体,moesin 在郎飞结处的施万细胞突起中表达,对髓鞘形成至关重要。在 40 例 GBS 患者中,有 6 例患者近期发生 CMV 感染,其中 5 例(83%)患者血清中 moesin 免疫球蛋白 G 抗体水平较高。在包括 5 例有 CMV 感染但无神经病的疾病对照者和仅 2 例(4%)正常对照者中均未发现抗 moesin 抗体。免疫细胞化学显示,CMV 相关 AIDP 患者的血清可在施万细胞瘤的远端尖端染色 moesin,但对照者的血清则不能。

结论

moesin 可能是 CMV 相关 AIDP 患者致病性自身抗体的免疫靶标分子。

证据分类

本研究提供了 II 级证据,表明血清抗 moesin 抗体水平可准确区分 CMV 相关 AIDP 与非 CMV 相关 AIDP(敏感性 83%,特异性 93%)。

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