...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Molecular and Cellular Biology >Hip1-related Mutant Mice Grow and Develop Normally but Have Accelerated Spinal Abnormalities and Dwarfism in the Absence of HIP1
【24h】

Hip1-related Mutant Mice Grow and Develop Normally but Have Accelerated Spinal Abnormalities and Dwarfism in the Absence of HIP1

机译：Hip1相关的突变小鼠正常生长和发育，但在没有HIP1的情况下具有加速的脊柱异常和侏儒症。

获取原文
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

In mice and humans, there are two known members of the Huntingtin interacting protein 1 (HIP1) family, HIP1 and HIP1-related (HIP1r). Based on structural and functional data, these proteins participate in the clathrin trafficking network. The inactivation of Hip1 in mice leads to spinal, hematopoietic, and testicular defects. To investigate the biological function of HIP1r, we generated a Hip1r mutant allele in mice. Hip1r homozygous mutant mice are viable and fertile without obvious morphological abnormalities. In addition, embryonic fibroblasts derived from these mice do not have gross abnormalities in survival, proliferation, or clathrin trafficking pathways. Altogether, this demonstrates that HIP1r is not necessary for normal development of the embryo or for normal adulthood and suggests that HIP1 or other functionally related members of the clathrin trafficking network can compensate for HIP1r absence. To test the latter, we generated mice deficient in both HIP1 and HIP1r. These mice have accelerated development of abnormalities seen in Hip1 -deficient mice, including kypholordosis and growth defects. The severity of the Hip1r/Hip1 double-knockout phenotype compared to the Hip1 knockout indicates that HIP1r partially compensates for HIP1 function in the absence of HIP1 expression, providing strong evidence that HIP1 and HIP1r have overlapping roles in vivo.
机译：在小鼠和人类中，Huntingtin相互作用蛋白1（HIP1）家族有两个已知成员，即HIP1和HIP1相关（HIP1r）。根据结构和功能数据，这些蛋白质参与网格蛋白运输网络。小鼠 Hip1 失活会导致脊髓，造血和睾丸缺陷。为了研究HIP1r的生物学功能，我们在小鼠中生成了一个 Hip1r 突变等位基因。 Hip1r 纯合突变小鼠存活且可育，没有明显的形态异常。另外，衍生自这些小鼠的胚胎成纤维细胞在存活，增殖或网格蛋白运输途径中没有明显异常。总之，这表明HIP1r对于胚胎的正常发育或正常成年不是必需的，并表明HIP1或网格蛋白贩运网络中其他功能相关的成员可以弥补HIP1r的缺失。为了测试后者，我们生成了同时缺乏HIP1和HIP1r的小鼠。这些小鼠加速了在 Hip1 缺陷小鼠中出现的异常发展，包括驼背病和生长缺陷。与 Hip1 敲除相比， Hip1r / Hip1 双敲除表型的严重性表明，在没有HIP1的情况下，HIP1r可以部分补偿HIP1的功能。表达，提供有力的证据表明HIP1和HIP1r在体内具有重叠的作用。

著录项

相似文献

  • 外文文献
  • 中文文献
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