Johnston I, Lang B, Leys K, Newsom-Davis J
Institute of Molecular Medicine, University of Oxford, John Radcliff Hospital, UK.
Neurology. 1994 Feb;44(2):334-8. doi: 10.1212/wnl.44.2.334.
We investigated the heterogeneity of anti-voltage-gated calcium channel (VGCC) antibodies in the Lambert-Eaton myasthenic syndrome (LEMS) using a small-cell lung carcinoma line (MB) derived from an LEMS patient. Four of 13 LEMS patients had raised titers of anti-125I-omega-conotoxin-labeled (N-type) VGCCs, measured by radioimmunoassay using line MB as the source of antigen. Antagonists for L-type (nitrendipine and nifedipine) and N-type (omega-conotoxin) VGCCs inhibited K(+)-stimulated (voltage-dependent) Ca2+ flux into this line--by 22% for L-type and 2% for N-type at maximum concentration. Inhibition by the LEMS IgGs, by contrast, ranged from 46 to 78% at a concentration of 2 mg/ml. These differing effects on Ca2+ flux inhibition by LEMS IgGs on the one hand and by L- and N-type channel antagonists on the other, taken together with the observation that many of the sera failed to react with omega-conotoxin-labeled (N-type) channels in the immunoprecipitation assay, suggest that in many LEMS patients the autoantibodies target other VGCC subtypes besides L- or N-types, and that these are important in inducing the myasthenic disorder.
我们使用源自一名兰伯特-伊顿肌无力综合征(LEMS)患者的小细胞肺癌细胞系(MB),研究了LEMS中抗电压门控钙通道(VGCC)抗体的异质性。在13例LEMS患者中,有4例患者通过以细胞系MB作为抗原来源的放射免疫分析法,检测出抗125I-ω-芋螺毒素标记(N型)VGCC的滴度升高。L型(尼群地平和硝苯地平)和N型(ω-芋螺毒素)VGCC的拮抗剂抑制K⁺刺激(电压依赖性)Ca²⁺流入该细胞系——最大浓度时,L型拮抗剂抑制22%,N型拮抗剂抑制2%。相比之下,LEMS免疫球蛋白G(IgG)在浓度为2mg/ml时的抑制率为46%至78%。一方面LEMS IgG对Ca²⁺流入抑制的作用与L型和N型通道拮抗剂的作用不同,另一方面,结合免疫沉淀试验中许多血清未能与ω-芋螺毒素标记(N型)通道发生反应这一观察结果,表明在许多LEMS患者中,自身抗体除了靶向L型或N型之外,还靶向其他VGCC亚型,并且这些亚型在诱发肌无力疾病中很重要。