...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>The European Journal of Neuroscience >Vascular changes in the cerebellum of Norrin /Ndph knockout mice correlate with high expression of Norrin and Frizzled-4.
【24h】

Vascular changes in the cerebellum of Norrin /Ndph knockout mice correlate with high expression of Norrin and Frizzled-4.

机译：Norrin / Ndph基因敲除小鼠小脑的血管变化与Norrin和Frizzled-4的高表达有关。

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

X-linked Norrie disease, familial exudative vitreoretinopathy (FEVR), Coat's disease and retinopathy of prematurity are severe human eye diseases and can all be caused by mutations in the Norrie disease pseudoglioma gene. They all show vascular defects and characteristic features of retinal hypoxia. Only Norrie disease displays additional neurological symptoms, which are sensorineural hearing loss and mental retardation. In the present study, we analysed transcript levels of the ligand Norrin (Ndph) and its two receptors Frizzled-4 (Fzd4) and LDL-related protein receptor 5 (Lrp5) in six different brain regions (cerebellum, cortex, hippocampus, olfactory bulb, pituitary and brain stem) of 6- to 8-month-old wild-type and Ndph knockout mice by quantitative real-time PCR. No effect of the Ndph knockout allele on Fzd4 or Lrp5 receptor expression was found. Furthermore, no alterations of the transcript levels of three hypoxia-regulated angiogenic factors (Vegfa, Itgrb3 and Tie1) were observed in the absence of Norrin. Interestingly, we identified significant differences in Ndph, Fzd4 and Lrp5 transcript levels in brain regions of wild-type mice and observed highest expression of Norrin and frizzled-4 in cerebellum. Transcript analyses were correlated with morphological data obtained from cerebellum and immunohistochemical studies of blood vessels in different brain regions. Vessel density was reduced in the cerebellum of Ndph knockout mice but the number of Purkinje and granular cells was not altered. This provides the first description of a brain phenotype in Ndph knockout mice, which will help to elucidate the role of Norrin in the brain.
机译：X联结的诺里氏病，家族性渗出性玻璃体视网膜病变（FEVR），科茨氏病和早产儿视网膜病是严重的人眼疾病，都可能由诺里氏病假神经胶质瘤基因突变引起。它们都显示出血管缺损和视网膜缺氧的特征。仅诺里氏病显示其他神经系统症状，即感觉神经性听力减退和智力低下。在本研究中，我们分析了六个不同大脑区域（小脑，皮层，海马，嗅球）中配体Norrin（Ndph）及其两个受体Frizzled-4（Fzd4）和LDL相关蛋白受体5（Lrp5）的转录水平，垂体和脑干）通过实时定量PCR检测6至8个月大的野生型和Ndph基因敲除小鼠。未发现Ndph敲除等位基因对Fzd4或Lrp5受体表达的影响。此外，在没有Norrin的情况下，未观察到三种低氧调节的血管生成因子（Vegfa，Itgrb3和Tie1）的转录水平没有变化。有趣的是，我们在野生型小鼠的大脑区域中发现了Ndph，Fzd4和Lrp5转录水平的显着差异，并观察到小脑中Norrin和frizzled-4的最高表达。转录分析与从小脑获得的形态学数据和不同脑区血管的免疫组织化学研究相关。 Ndph基因敲除小鼠小脑的血管密度降低，但Purkinje和颗粒细胞的数量没有改变。这提供了对Ndph基因敲除小鼠的大脑表型的首次描述，这将有助于阐明Norrin在大脑中的作用。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号