瞬时受体电位通道蛋白3在结节性硬化症癫痫患者皮层病灶区中的表达  

Expression of TRPC3 in the cortical focus of epilepsy patients with tuberous sclerosis complex

作　　者：赵湧频 陈康宁[1] 树海峰[3] 余思逊[3] 杨辉[3] 

机构地区：[1]第三军医大学西南医院神经内科,重庆400038 [2]第一人民医院神经内科,贵阳550002 [3]第三军医大学新桥医院神经外科,全军癫痫病诊治中心,重庆400037

出　　处：《第三军医大学学报》2012年第10期983-986,共4页Journal of Third Military Medical University

摘　　要：目的研究瞬时受体电位通道蛋白3(transient receptor potential canonical channel 3,TRPC3)在结节性硬化症(tuberous sclerosis complex,TSC)癫痫患者皮层脑组织的表达与分布,探讨TRPC3离子通道参与结节性硬化致癫痫发作的可能机制。方法收集自2008年6月至2010年12月手术切除并经病理检测证实是TSC癫痫患者的皮层脑组织标本12例,与7例正常对照皮层脑组织比较,通过采用Western blot、免疫组化、免疫荧光双标,检测TRPC3通道蛋白在正常脑组织与病理标本中的表达分布情况。结果 Western blot蛋白检测结果显示,TRPC3在TSC病理标本中的相对光密度值(ROD)为(0.65±0.08),在正常脑组织(CTX)中的ROD值为(0.36±0.06),提示TRPC3在TSC标本中的表达较正常脑组织中显著增高(P<0.05);免疫组化及免疫荧光双标结果,TRPC3在TSC病灶中免疫强度明显增高,并且特异性高表达于皮层损伤区致痫灶中的异构神经元,包括特征性的巨型细胞(giant cells,GCs)、异形神经元(dysplastic neurons,DNs)。结论 TRPC3在结节性硬化症患者致痫皮层中表达异常增高,以及特异性的细胞分布模式可能与癫痫发病密切相关。Objective To investigate the expression and distribution of transient receptor potential canonical channel 3（TRPC3） in the cortical focus of epilepsy patients with tuberous sclerosis complex（TSC）,and to explore the mechanism of TRPC3 involvement in TSC-induced epileptic seizure.Methods Twelve cases of TSC epileptic brain tissues（TSC group） confirmed by pathological examination from June 2008 to December 2010 and seven cases of non-epileptic brain tissues（CTX group） were enrolled in our study.The expression and distribution of TRPC3 were observed by Western blotting,immunohistochemistry and immunofluorescence double labeling in these tissues.Results Western blot analysis showed that the TRPC3 expression was significantly higher in the TSC group than in the CTX group [relative optical density（ROD） 0.65±0.08 vs 0.36±0.06,P0.05].The immunohistochemistry and immunofluorescence double labeling results showed that the intensity score of TRPC3 was higher in the TSC group than in the CTX group,and TRPC3 was specifically overexpressed in the misshapen neurons of the cortical focus,including dysplastic neurons（DNs） and giant cells（GCs）.Conclusion The abnormally increased expression of TRPC3 in epileptic cortex of TSC patients and the specific cell distribution pattern may be closely involved in the pathogenesis of intractable epilepsy.

关 键 词：TRPC3 结节性硬化症(TSC) 癫痫 

分 类 号：R741.02[医药卫生—神经病学与精神病学] R749.17[医药卫生—临床医学]