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首页> 外文期刊>Neurochemistry International: The International Journal for the Rapid Publication of Critical Reviews, Preliminary and Original Research Communications in Neurochemistry >Spastin in the human and mouse central nervous system with special reference to its expression in the hippocampus of mouse pilocarpine model of status epilepticus and temporal lobe epilepsy.
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Spastin in the human and mouse central nervous system with special reference to its expression in the hippocampus of mouse pilocarpine model of status epilepticus and temporal lobe epilepsy.

机译：Spastin在人和小鼠的中枢神经系统中，特别提及其在状态为癫痫和颞叶癫痫的小鼠毛果芸香碱模型海马中的表达。

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摘要

In the present in situ hybridization and immunocytochemical studies in the mouse central nervous system (CNS), a strong expression of spastin mRNA and protein was found in Purkinje cells and dentate nucleus in the cerebellum, in hippocampal principal cells and hilar neurons, in amygdala, substantia nigra, striatum, in the motor nuclei of the cranial nerves and in different layers of the cerebral cortex except piriform and entorhinal cortices where only neurons in layer II were strongly stained. Spastin protein and mRNA were weakly expressed in most of the thalamic nuclei. In selected human brain regions such as the cerebral cortex, cerebellum, hippocampus, amygdala, substania nigra and striatum, similar results were obtained. Electron microscopy showed spastin immunopositive staining in the cytoplasma, dendrites, axon terminals and nucleus. In the mouse pilocarpine model of status epilepticus and subsequent temporal lobe epilepsy, spastin expression disappeared in hilar neurons as early as at 2h duringpilocarpine induced status epilepticus, and never recovered. At 7 days and 2 months after pilocarpine induced status epilepticus, spastin expression was down-regulated in granule cells in the dentate gyrus, but induced expression was found in reactive astrocytes. The demonstration of widespread distribution of spastin in functionally different brain regions in the present study may provide neuroanatomical basis to explain why different neurological, psychological disorders and cognitive impairment occur in patients with spastin mutation. Down-regulation or loss of spastin expression in hilar neurons may be related to their degeneration and may therefore initiate epileptogenetic events, leading to temporal lobe epilepsy.
机译：在小鼠中枢神经系统（CNS）的当前原位杂交和免疫细胞化学研究中，在杏仁，小脑，海马主要细胞和肺门神经元的Purkinje细胞和齿状核中发现了spastin mRNA和蛋白的强表达，黑质，纹状体，在颅神经的运动核中和在大脑皮层的不同层中，除了梨状和内嗅皮层（仅II层的神经元被强烈染色）。 Spastin蛋白和mRNA在大多数丘脑核中表达较弱。在选定的人类大脑区域，如大脑皮层，小脑，海马，杏仁核，黑质和纹状体，获得了相似的结果。电镜显示胞质，树突，轴突末端和细胞核中的spastin免疫阳性染色。在癫痫持续状态和随后的颞叶癫痫的小鼠毛果芸香碱模型中，早在毛果芸香碱引起的癫痫持续状态的2小时内，spastin表达就在肺门神经元中消失了，并且从未恢复。毛果芸香碱诱发癫痫持续状态后第7天和2个月，齿状回颗粒细胞中的spastin表达下调，但反应性星形胶质细胞中发现诱导表达。这项研究表明，spastin在功能不同的大脑区域广泛分布，这可能为解释为什么spastin突变的患者发生不同的神经，心理障碍和认知障碍提供神经解剖学基础。肺门神经元中spastin表达的下调或丧失可能与它们的变性有关，因此可能引发癫痫发生事件，导致颞叶癫痫。

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