TRPC6在结节性硬化症癫痫患者皮层脑组织中的表达

Expression of TRPC6 in the cortical focus of patients with epilepsy from tuberous sclerosis complex

目的研究瞬时受体电位通道蛋白C6(TRPC6)在结节性硬化症(TSC)癫痫患者皮层脑组织的表达与分布,探讨TRPC6通道参与结节性硬化致痫的可能机制。方法收集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18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TRPC6在正常脑组织与TSC病理标本中的表达分布情况。结果 Western blot蛋白检测结果显示,在TSC和对照大脑皮质(CTX)TRPC6均在相应的分子量94KDa处有特异性蛋白条带,与CTX比较,TSC组TRPC6条带与内参GAPDH条带灰度值之比显著增高(P<0.05);免疫组化及免疫荧光双标结果显示,TRPC6在TSC病灶中免疫强度明显增高,且特异性高表达于皮层损伤区致痫灶中的异构神经元,包括特征性的巨形细胞(GCs)、异形神经元(DNs)。结论 TRPC6在结节性硬化症患者致痫皮层脑组织中表达异常增高,特异性的细胞分布模式可能与癫痫发病密切相关。

Objective To detect the expression of TRPC6 in the cortical lesions of patients with epilepsy from tuberous sclerosis complex.Methods 18 clinical cases of TSC and 7cases of non-epileptic brain tissues were enrolled in our study,and the expression pattern of TRPC6 was observed by immunohistochemistry and western blot（WB）analysis.Results WB analysis result was showed that TRPC6 expression tended to be higher in TSC compared with CTX immunohistochemistry and immune fluorescent double labeling analysis showed that immune strength of TRPC6 tended to be higher in TSC compared with CTX and TRPC6 was moderate-strong expressed in misshapen cells,including dysmorphic neurons（DNs）and giant cells（GCs）.Conclusion The increased expression of TRPC6 in epileptic cortex might be closely involved in the pathogenesis of intractable epilepsy.