中文摘要：

目的研究瞬时受体电位通道蛋白5（transient receptor potential canonical channel 5,TRPC5）在结节性硬化症（tuberous sclerosis complex,TSC）癫痫患者皮质中的表达与分布,探讨TRPC5在TSC癫痫发生中的可能机制。方法收集新桥医院神经外科手术切除的TSC癫痫患者皮层致痫结节14例,与12例正常对照皮层（CTX）相比较,运用RT-PCR、Western blot、免疫组织化学和免疫荧光等方法检测TRPC5在致痫灶中的表达分布。结果 TSC癫痫患者皮层致痫结节中,TRPC5在mRNA、蛋白表达水平较对照组明显升高（P〈0.05）。免疫组化结果显示,在正常皮层中TRPC5弱至中等强度表达于神经元上,偶在胶质细胞上表达,而在TSC癫痫患者皮层致痫结节中强表达于神经元上,特别是巨型细胞和异形神经元上,胶质细胞表达强度也较CTX组增高,免疫染色强度明显高于正常皮层（染色强度值：2.77±0.43 vs 1.64±0.56;相对光密度值：64.72±9.56 vs 30.34±9.43;P〈0.05）。结论 TRPC5表达增高可能与TSC患者癫痫发病密切相关,可能成为治疗难治性癫痫的新药物靶点。

英文摘要：

Objective To investigate the expression and distribution of transient receptor potential canonical channel 5（TRPC5） in the cortical tubers of epilepsy patients with tuberous sclerosis complex（TSC）,and to explore the mechanism of TRPC5 involved in TSC-induced epileptic seizure.Methods Fourteen cases of TSC epileptic cortical tubers（TSC group） confirmed by pathological examination and twelve cases of non-epileptic cortices（CTX group） were collected.The expression and distribution of TRPC5 in the samples were examined by RT-PCR,Western blotting,immunohistochemical and double-labeling immunofluorescencestaining.Results The mRNA and protein levels of TRPC5 were significantly higher in the TSC group than those in the CTX group（P0.05）.The immunohistochemical results showed that TRPC5 was weakly or moderately expressed in the neurons and occasionally expressed in the glial cells in the CTX group.However,strong expression of TRPC5 was observed in the neurons,glial cells,dysplastic neurons（DNs） and giant cells（GCs） in the TSC group.The intensity of immunofluorescence was significantly higher in the TSC group than in the CTX group（intensity score： 2.77±0.63 vs 1.64±0.66;ratio of mean optical density 44.72±8.56 vs 30.34±9.43;P0.05）.Conclusion The up-regulation of TRPC5 in the cortical tubers of epilepsy patients with TSC may be closely associated with the pathogenesis of epilepsy,suggesting that TRPC5 might be a new potential drug target for the treatment of intractable epilepsy.