重症肌无力患者肌细胞分拣微管连接蛋白17的表达及作用  

Expression and function of sorting nexin 17 in myasthenia gravis muscle cells

作　　者：朱明振[1] 张婧[3] 张迎娜[4] 吕杰[3] 赵雪[3] 方华[3] 崔新征[2] 张清勇[2] 高峰[3] 

机构地区：[1]郑州大学第二附属医院神经内科,450014 [2]河南省医药科学研究院神经免疫学研究室 [3]郑州大学基础医学院微生物与免疫学教研室 [4]郑州大学第二附属医院普胸外科,450014

出　　处：《中华实验外科杂志》2015年第11期2754-2757,共4页Chinese Journal of Experimental Surgery

基　　金：河南省重点科技攻关计划资助项目（122102310055、122102310237）;郑州市技术研究与开发项目（10PTGS482）;河南省教育厅科学技术研究重点项目（12A320028、12A320066）

摘　　要：目的 比较分拣微管连接蛋白17（SNX17）在重症肌无力（MG）组和对照组肋间肌肌细胞中的表达,探讨其在乙酰胆碱受体（AChR）聚集中的作用.方法 取MG组和对照组肋间肌,分离出单根肌丝,用免疫荧光法观察肌丝AChR、SNX17分子形态及分布;实时荧光定量聚合酶链反应（FQ-PCR）、Western blot法比较两组肌细胞SNX17含量.结果 AChR位于肌纤维纵轴的中部,呈连续光滑的红色椭圆形突起.对照组AChR呈曲奇饼干样,内部有精细分支的网络状结构;MG组仅可观察到呈不连续完整的红色外周轮廓线,内部网状结构破碎,呈空泡样,但可观察到SNX17呈同样形状蓝色椭圆形突起,两者位置、形态一致.MG组SNX17 mRNA和蛋白含量（n=10,0.85±0.08;0.83 ±0.08）均明显高于对照组（n=14,0.57±0.09;0.65±0.06）,差异有统计学意义（t=5.75 ,P ＜0.05;t=3.03 ,P ＜0.05）.结论 MG患者肋间肌肌细胞SNX17表达增高,与AChR在神经肌肉接头处共定位,可能是促进AChR聚集,维护神经肌肉接头信号通路完整的重要分子.Objective By comparing sorting nexin 17 （SNX17） expression in intercostal muscle cells in myasthenia gravis （MG） group and control group, we study the role of SNX17 in the aggregation of acetylcholine receptors （AchRs）.Methods Muscles were separated into a single cell.We used immunofluorescence to observe the form of acetylcholine receptor and SNX17 and compare the content of SNX17 in intercostal muscle cells by real-time fluorescent quantitative polymerase chain reaction （FQ-PCR） and Western blotting between the two groups.Results The AChRs was located in the central part in the vertical axis of muscle fiber, a continuous smooth red oval bump.The AChRs outer looked like cookies with branch of internal fine reticular structure in control group.In MG group, only red outer contour, internal network structure, could be observed, with the cavitation, but that could be observed in the same blue oval shape.The location and shape were the same.The expression of SNX17 in MG group （n =10, 0.85 ± 0.08;0.83 ± 0.08） was significantly higher than in control group （n =14, 0.57 ± 0.09;0.65 ± 0.06） by Western blotting and FQ-PCR.The difference was statistically significant （t =5.75, P 〈 0.05;t =3.03, P 〈0.05）.Conclusion SNX17 in intercostal muscle cells is over-expressed in MG group, and positioned in the neuromuscular junction with AChRs.The SNX17 may play an important role in the neuromuscular junction structure and promote the aggregation of AChRs.

关 键 词：分拣微管连接蛋白17 重症肌无力 乙酰胆碱受体 神经肌肉接头 

分 类 号：R746.1[医药卫生—神经病学与精神病学]