Abnormal features in mutant cerebellar Purkinje cells lacking junctophilins
Abnormal features in mutant cerebellar Purkinje cells lacking junctophilins
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DOI：
10.1016/j.bbrc.2007.09.062
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发表时间：
2007-11-23
影响因子：
3.1
通讯作者：
Takeshima, Hiroshi
中科院分区：
文献类型：
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作者：
Ikeda, Atsushi;Miyazaki, Taisuke;Takeshima, Hiroshi
连接蛋白产生的连接膜复合物（JMCs）是可兴奋细胞中Ca（2+）介导的细胞表面和细胞内通道之间的通讯所必需的。敲除小鼠缺乏神经嗜连接蛋白（JP-DKO）显示严重的运动缺陷和不规则的小脑可塑性，由于取消通道串扰浦肯野细胞（PC）。为了精确地理解JP-DKO小鼠中的畸变，我们进一步分析了突变PC。在通过电刺激诱导小脑可塑性的过程中，JP-DKO PC表现出不充分的去极化反应。免疫化学检测到JP-DKO PC中突触成熟和蛋白激酶C γ过度磷酸化的轻度损伤。此外，基因表达略有改变，在JP-DKO小脑。因此，突变PC具有边缘但广泛的异常，所有这些都可能导致JP-DKO小鼠的小脑运动缺陷。(C)2007年爱思唯尔公司All rights reserved.
Junctional membrane complexes (JMCs) generated by junctophilins are required for Ca (2+) -Mediated communication between cell-surface and intracellular channels in excitable cells. Knockout mice lacking neural junctophilins (JP-DKO) show severe motor defects and irregular cerebellar plasticity due to abolished channel crosstalk in Purkinje cells (PCs). To precisely understand aberrations in JP-DKO mice, we further analyzed the mutant PCs. During the induction of cerebellar plasticity via electrical stimuli, JP-DKO PCs showed insufficient depolarizing responses. Immunochemistry detected mild impairment in synaptic maturation and hyperphosphorylation of protein kinase C gamma in JP-DKO PCs. Moreover, gene expression was slightly altered in the JP-DKO cerebellum. Therefore, the mutant PCs bear marginal but widespread abnormalities, all of which likely cause cerebellar motor defects in JP-DKO mice. (C) 2007 Elsevier Inc. All rights reserved.