Altered distribution of KCC2 in cortical dysplasia in patients with intractable epilepsy
Altered distribution of KCC2 in cortical dysplasia in patients with intractable epilepsy
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DOI：
10.1111/j.1528-1167.2006.00954.x
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发表时间：
2007-04-01
期刊：
影响因子：
5.6
通讯作者：
Tsuchiya, Shigeru
中科院分区：
文献类型：
--
作者：
Munakata, Mitsutoshi;Watanabe, Mika;Tsuchiya, Shigeru
目的：研究神经元特异性K+- cl -共转运蛋白KCC2在人皮质发育不良（CD）中的分布。方法：对18例癫痫手术CD标本中KCC2的免疫组化表达进行研究。组织病理学诊断为局灶性CD (FCD) I型（8例）、FCD II型（6例）和半巨脑畸形（HME） 4例。对组织切片进行KCC2免疫染色，并与对照切片进行比较。结果：成熟非发育不良皮质各层均可见弥漫性KCC2染色。尽管皮质下异位神经元在胞浆中显示密集的染色（胞内染色），但体细胞染色少得多。在FCD I型中，皮层显示KCC2的neuropil染色，而体细胞染色较少。异常的巨锥体神经元在胞内染色较少，而未成熟的神经元在胞内染色。皮层下白质中胞内染色的异位神经元增多。在FCD II型中，畸形神经元显示致密的胞内染色，邻近的神经丸染色减少。球囊细胞未见KCC2染色。HME中畸形神经元也有胞内染色。结论：CD组织神经元表达KCC2。然而，KCC2的亚细胞分布发生了改变，这可能影响了CD组织中参与癫痫活动的Cl-和K+的离子稳态。
Purpose: To examine the distribution of KCC2, a neuron-specific K+-Cl- cotransporter, in human cortical dysplasia (CD).Methods: The immunohistochemical expression of KCC2 was investigated in 18 CD specimens obtained during epilepsy surgery. The histopathologic diagnoses were focal CD (FCD) type I (eight cases), FCD type II (six cases), and hemimegalencephaly (HME; four cases). Tissue sections were immunostained for KCC2 and compared with control sections.Results: In the mature nondysplastic cortex, all the layers showed diffuse neuropil staining for KCC2. The somata were stained much less, although subcortical ectopic neurons displayed dense staining in the cytosol (intrasomatic staining). In FCD type I, the cortex showed neuropil staining for KCC2 with less-stained somata. Aberrant giant pyramidal neurons were also less stained at the soma, whereas immature neurons showed intrasomatic staining. Increased numbers of ectopic neurons with intrasomatic staining were noted in the subcortical white matter. In FCD type II, dysmorphic neurons displayed dense intrasomatic staining with reduced staining of the neighboring neuropils. Balloon cells did not stain for KCC2. Dysmorphic neurons in HME also showed intrasomatic staining.Conclusions: Neurons in CD tissues expressed KCC2. However, the subcellular distribution of KCC2 was altered, which might have affected the ionic homeostasis of Cl- and K+ involved in epileptic activity within CD tissues.