兰伯特-伊顿肌无力综合征中针对重组突触结合蛋白和钙通道亚型的抗体。

Antibodies to recombinant synaptotagmin and calcium channel subtypes in Lambert-Eaton myasthenic syndrome.

作者信息

Takamori M, Takahashi M, Yasukawa Y, Iwasa K, Nemoto Y, Suenaga A, Nagataki S, Nakamura T

机构信息

Department of Neurology, Kanazawa University School of Medicine, Ishikawa-ken, Japan.

出版信息

J Neurol Sci. 1995 Nov;133(1-2):95-101. doi: 10.1016/0022-510x(95)00162-u.

DOI:10.1016/0022-510x(95)00162-u

PMID:8583238

Abstract

Several proteins have been postulated as possible targets of immune attack in Lambert-Eaton myasthenic syndrome (LEMS). Heterogeneity of autoantibodies in sera from 20 LEMS patients was studied by comparing their reactivity to synaptotagmin, a synaptic vesicle protein, and voltage-gated calcium channels (VGCCs). Six patients' sera (1 with small cell lung carcinoma (SCLC)) contained antibodies specifically recognizing the recombinant synaptotagmin on immunoblots. Thirteen (11 with SCLC) and 16 (11 with SCLC and 1 with poorly differentiated cell carcinoma in the lung) patients' sera immunoprecipitated omega-conotoxin GVIA-labeled N-type and omega-conotoxin MVIIC-labeled Q-type VGCCs, respectively. Three of 6 synaptotagmin-positive sera had cross-reactivity with N and/or Q subtypes of VGCC; the remaining 3 showed no cross-reactivity with VGCCs. Results indicate that LEMS sera are heterogeneous in the spectrum of containing antibodies, and suggest that this heterogeneity reflects the immune response to various synaptic proteins including not only multiple VGCCs but also synaptosecretory complex proteins.

摘要

在兰伯特-伊顿肌无力综合征（LEMS）中，有几种蛋白质被假定为可能的免疫攻击靶点。通过比较20例LEMS患者血清中的自身抗体与突触囊泡蛋白突触结合蛋白以及电压门控钙通道（VGCCs）的反应性，研究了这些自身抗体的异质性。6例患者（1例合并小细胞肺癌（SCLC））的血清在免疫印迹中含有特异性识别重组突触结合蛋白的抗体。13例（11例合并SCLC）和16例（11例合并SCLC和1例合并肺低分化细胞癌）患者的血清分别免疫沉淀了ω-芋螺毒素GVIA标记的N型和ω-芋螺毒素MVIIC标记的Q型VGCCs。6例突触结合蛋白阳性血清中有3例与VGCC的N和/或Q亚型有交叉反应；其余3例与VGCCs无交叉反应。结果表明，LEMS血清中的抗体谱具有异质性，并提示这种异质性反映了对包括多种VGCCs以及突触分泌复合物蛋白在内的各种突触蛋白的免疫反应。