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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 cortex brain tissue in patients with epilepsy of tuberculous sclerosis complex (TSC), and to explore the role of TRPC3 ion channel in nodular Possible mechanism of sclerosis caused by seizures. Methods Twelve cases of cortical brain tissue were collected from June 2008 to December 2010 and were confirmed by pathological examination. Compared with 7 normal control cortical brain tissue samples, Western blot, immunohistochemistry, Immunofluorescence double labeling, detection of TRPC3 channel protein expression in normal brain tissue and pathological specimens. Results The results of Western blot showed that the relative optical density (ROD) of TRPC3 in TSC was (0.65 ± 0.08) and that in normal brain tissue (CTX) was (0.36 ± 0.06), suggesting that TRPC3 was The expression of TRPC3 in TSC was significantly higher than that in normal brain tissue (P <0.05). Immunohistochemistry and immunofluorescence double-labeled results showed that the TRPC3 immunostaining was significantly increased in TSC lesions and was highly expressed in epileptogenic lesions Heterogeneous neurons, including characteristic giant cells (GCs), dysplastic neurons (DNs). Conclusion TRPC3 expression in epileptogenic cortex of patients with tuberous sclerosis abnormally increased, and specific cell distribution patterns may be closely related with the incidence of epilepsy.