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朗伯综合征 ( LEMS)常与小细胞性肺癌 ( SCLC)伴发 ,是一种神经肌肉接头处兴奋传导障碍性疾病 ,其运动神经末梢抗体直接抑制电门控式钙通道 ( VGCC) ,引起乙酰胆碱( Ach)释放量减少起着关键作用。人们注意到 LEMS病人体内有特殊的抗体 ,主要针对 VGCC中 P/Q亚型的钙通道 ,而且 P/Q亚型 VGCC在 SCLC病人中也有表达 ,故运动神经末梢和SCLC细胞可能具有共同的 VGCC抗原性。为了在分子水平上寻找抗原位点 ,有人使用VGCCα1 A亚单位中 4个区域的 S5 - S6连接部位的多肽或重组蛋白来研究它们的抗原性 ,结果显示通过免疫诱导 LEMS动物模型及测定 LEMS病人体内抗体 ,认定其 、 、 域是免疫显性部位 ;同时用相当与突触结合蛋白 1的多肽和重组蛋白进行实验 ,发现这种 VGCC相关蛋白 ,其暴露在细胞外的部分在胞吐过程中对 LEMS具有抗原性。
Lambert syndrome (LEMS) often associated with small cell lung cancer (SCLC) is a neuromuscular junction excitability conduction disorder, the motor nerve endothelin directly inhibit the electric gated calcium channel (VGCC), caused Reduced release of acetylcholine (Ach) plays a key role. People have noticed that there are special antibodies in LEMS patients, mainly targeting calcium channels of P / Q subtype in VGCC, and P / Q subtype VGCC is also expressed in SCLC patients. Therefore, motor nerve endings and SCLC cells may have a common VGCC Antigenicity. In order to search for antigenic sites at the molecular level, the antigens were studied using peptides or recombinant proteins at the S5-S6 junction in the 4 regions of the VGCCα1 A subunit. The results showed that the animal model of LEMS was induced by immunization and the LEMS patient Antibodies in vivo were identified as immunodominant sites. At the same time, experiments with peptides and recombinant proteins corresponding to synaptotagmin-binding protein 1 revealed that this VGCC-associated protein, which is exposed in the extracellular part of the exocytosis process Antigenicity to LEMS.