Elevated Expression of TRPC4 in Cortical Lesions of Focal Cortical Dysplasia II and Tuberous Sclerosis Complex
Elevated Expression of TRPC4 in Cortical Lesions of Focal Cortical Dysplasia II and Tuberous Sclerosis Complex
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TRPC4 在局灶性皮质发育不良 II 和结节性硬化症的皮质病变中表达升高
DOI：
10.1007/s12031-017-0923-z
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发表时间：
2017-04
影响因子：
3.1
通讯作者：
Yang Hui
中科院分区：
文献类型：
--
作者：
Wang Lu Kang;Chen Xin;Zhang Chun Qing;Liang Chao;Wei Yu Jia;Yue Jiong;Liu Shi Yong;Yang Hui
局灶性皮质发育不良II型（FCD II）和结节性硬化症（TSC）是儿童慢性难治性癫痫的常见原因。经典瞬时受体电位通道（TRPC）是一种非选择性阳离子通道，通常由磷脂酶C（PLC）刺激激活。以往的研究发现TRPC 4可能参与了癫痫的发生过程。本研究旨在检测TRPC 4在FCD II（n= 24）和TSC（n= 11）手术标本中的表达和分布，并与年龄匹配的尸检对照样本（n= 12）进行比较。我们发现，与对照样品相比，FCD II和TSC样品中TRPC 4及其上游因子PLC delta 1（PLCD 1）的蛋白水平升高。免疫组化结果显示，TRPC 4在畸形神经元、气球样细胞和巨细胞中的表达较强。在异常神经元中也发现了上游因子PLCD 1的中度至强染色。此外，双重免疫荧光染色显示TRPC 4与谷氨酸能和GABA能神经元标记物共定位。总之，我们的研究结果表明，TRPC 4蛋白的过表达可能参与FCD II和TSC的癫痫发生。
Focal cortical dysplasia type II (FCD II) and tuberous sclerosis complex (TSC) are well-known causes of chronic refractory epilepsy in children. Canonical transient receptor potential channels (TRPCs) are non-selective cation channels that are commonly activated by phospholipase C (PLC) stimulation. Previous studies found that TRPC4 may participate in the process of epileptogenesis. This study aimed to examine the expression and distribution of TRPC4 in FCD II (n= 24) and TSC (n= 11) surgical specimens compared with that in age-matched autopsy control samples (n= 12). We found that the protein levels of TRPC4 and its upstream factor, PLC delta 1 (PLCD1), were elevated in FCD II and TSC samples compared to those of control samples. Immunohistochemistry assays revealed that TRPC4 staining was stronger in malformed cells, such as dysmorphic neurons, balloon cells and giant cells. Moderate-to-strong staining of the upstream factor PLCD1 was also identified in abnormal neurons. Moreover, double immunofluorescence staining revealed that TRPC4 was colocalised with glutamatergic and GABAergic neuron markers. Taken together, our results indicate that overexpression of TRPC4 protein may be involved in the epileptogenesis of FCD II and TSC.
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影响因子：
1.4
作者：
通讯作者：
--
影响因子：
3.5
作者：
S. Spiczak;H. Muhle;I. Helbig;C. D. Kovel;J. Hampe;V. Gaus;B. Koeleman;D. Lindhout;S. Schreiber;T. Sander;U. Stephani
通讯作者：
S. Spiczak;H. Muhle;I. Helbig;C. D. Kovel;J. Hampe;V. Gaus;B. Koeleman;D. Lindhout;S. Schreiber;T. Sander;U. Stephani
DOI：
10.1038/npre.2011.6577.1
发表时间：
2011-11
期刊：
Nature Precedings
影响因子：
--
作者：
K. R. Illig;Kristin C. Rasmus;Andrew L. Varnell;E. Ostertag;W. Klipec;Donald C. Cooper
通讯作者：
K. R. Illig;Kristin C. Rasmus;Andrew L. Varnell;E. Ostertag;W. Klipec;Donald C. Cooper
影响因子：
6.1
作者：
水口 雅
通讯作者：
水口 雅
DOI：
--
发表时间：
1993
期刊：
--
影响因子：
--
作者：
J. Engel
通讯作者：
J. Engel