...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Proceedings of the Nutrition Society >Molecular regulation of copper excretion in the liver
【24h】

Molecular regulation of copper excretion in the liver

机译：肝脏中铜排泄的分子调控

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

Cu is an essential nutrient that is required for a broad range of cellular and molecular processes. Mammals have efficient systems to control Cu homeostasis that operate at the level of controlling uptake, distribution, sequestration and excretion of Cu. The study of diseases associated with disturbed Cu homeostasis has greatly enhanced our understanding of the molecular mechanisms involved in Cu metabolism. In man the liver is responsible for excreting excess Cu from the body by means of biliary secretion. Wilson disease is a severe human disorder characterized by Cu accumulation in the liver as a result of a deficiency in biliary Cu secretion. This disorder is caused by mutations in the gene that encodes a Cu-transporting P-type ATPase (ATP7B). The MURR1 gene was identified recently, and it was hypothesized that this gene is also essential for biliary Cu excretion and is presumed to act downstream of ATP7B. MURR1 is mutated in canine Cu toxicosis, a disorder with phenotypic characteristics similar to those of Wilson disease. MURR1 encodes a protein that is of unknown function and is without detectable sequence homology to known proteins. MURR1 is readily detected in all tissues and cell types, suggesting that it may exhibit a pleiotropic function in different organs, which may or may not be exclusively linked to Cu homeostasis. The use of genetic, biochemical and genomic tools, as well as the development of appropriate models in organisms other than dog, will allow the elucidation of the molecular and cellular function of MURR1 in relation to hepatic Cu homeostasis and biliary Cu excretion.
机译：铜是多种细胞和分子过程所必需的基本营养素。哺乳动物具有控制铜稳态的有效系统，该系统在控制铜的吸收，分布，螯合和排泄的水平上运行。对与铜稳态失调有关的疾病的研究极大地增进了我们对涉及铜代谢的分子机制的理解。在人体内，肝脏负责通过胆汁分泌物从体内排出过量的铜。威尔逊氏病是一种严重的人类疾病，其特征是胆汁中铜的分泌不足导致肝脏中的铜积累。此疾病是由编码铜转运P型ATP酶（ATP7B）的基因突变引起的。 MURR1基因是最近发现的，据推测该基因对于胆汁铜的排泄也是必不可少的，并且推测它在ATP7B的下游起作用。 MURR1在犬铜中毒中发生了突变，该病的表型特征类似于威尔逊氏病。 MURR1编码的蛋白质功能未知，与已知蛋白质没有可检测的序列同源性。 MURR1很容易在所有组织和细胞类型中检测到，提示它可能在不同器官中表现出多效性功能，而该功能可能与或不与铜体内稳态有关。遗传，生化和基因组学工具的使用，以及在除狗以外的生物中开发适当的模型，将有助于阐明MURR1与肝铜稳态和胆汁铜排泄有关的分子和细胞功能。

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