期刊文献+

铜的摄取转运与其相关基因疾病的讨论 被引量:3

The Uptake and Transport of Copper and Its Related Genetic Diseases
下载PDF
导出
摘要 铜是人体必需微量元素,机体既可通过精确的调节使它在生物学反应中发挥有益的作用,又可防止这种元素对毒性自由基形成的促进效应。文章主要探讨铜的摄取代谢转运过程并讨论这种元素所导致的与铜代谢有关的人类遗传性疾病,包括Menkes综合征和Wilson病。这两个疾病的相关蛋白被认为是铜转运磷酸化ATP酶,以下介绍两种疾病的病因及一些临床症状和治疗手段。 Copper is essential trace elements, the body can make it through precise regulation of the biological response play a useful role, to prevent such elements to promote the formation of toxic effects of free radicals. In the paper, the uptake of copper metabolism transit and discussed elements such as lead and copper metabolism of the human genetic diseases, including Menkes syndrome and Wilson disease. These two disease-related proteins are considered transit copper Phosphorylation of ATP, following on the two diseases and the cause of some of the clinical symptoms and treatment.
出处 《广东化工》 CAS 2016年第12期97-100,共4页 Guangdong Chemical Industry
基金 2015年海南省自然科学基金20152038
关键词 转运 WILSON病 Menkes综合征 铜转运磷酸化ATP酶 copper transit Wilson disease Menkesdisease Copper transit phosphorylation ATP
  • 相关文献

参考文献24

  • 1Stuehler B, Reichert J, Stremmel W, et al. Analysis of the human homologne of the canine copper toxicosis gene MURR1 in Wilson disease patients. J MolMed, 2004, 82: 629-634.
  • 2Bibudhendra Sarkar. Treatment of Wilson and Menkes Diseases. Chem Rev, 1999, 99.. 2535-2544.
  • 3Harris ED. Basic and clinical aspects of copper. Crit Rev Clin Lab Sci, 2003, 40.. 547-586.
  • 4Shim H, Harris ZL. Genetic defects in copper metabolism. J Nutr, 2003, 133: 1527S-15231S.
  • 5Gollan JL, Gollan TJ. Wilson disease in 1998: genetic, diagnostic and therapeutic aspects. JHepatol, 1998, 28: 28-36.
  • 6Ame-noF, BalatriE, BanciL, etal. Folding studiesofCox7revealan important interplay of cysteine oxidation and copper binding. Structure, 2005, 13: 713-722.
  • 7Svetlana L, Natalie L Barnes, Mee Y BARTEE, et al. Function and Regulation of Human Copper-Transporting ATPases. Physiol Rev, 2007, 87: 1011-1046.
  • 8Bryan T. Op't Holt and Kenneth M. Merz Jr. Insights into Cu(1) Exchange in HAH 1 Using Quantum Mechanical and Molecular. Biochemistry 2007, 46, 8816-8826.
  • 9Matthew H Sazinsky, Sorabh Agarwal, Jose" M Argu"ello, et al. Biochemistry, 2006, 45(33).
  • 10Cox D W, Moore S D. Copper transporting P-type ATPases and human disease, J BioenergBiomembr, 2002. 34: 333-338.

二级参考文献1

同被引文献29

引证文献3

二级引证文献11

相关作者

内容加载中请稍等...

相关机构

内容加载中请稍等...

相关主题

内容加载中请稍等...

浏览历史

内容加载中请稍等...
;
使用帮助 返回顶部