Hordyjewska Anna, Popiołek Łukasz, Kocot Joanna
Chair and Department of Medical Chemistry, Faculty of Medicine, Medical University of Lublin, 4A Chodzki Street, 20-093, Lublin, Poland.
Biometals. 2014 Aug;27(4):611-21. doi: 10.1007/s10534-014-9736-5. Epub 2014 Apr 20.
Copper (Cu) is an essential microelement found in all living organisms with the unique ability to adopt two different redox states-in the oxidized (Cu(2+)) and reduced (Cu(+)). It is required for survival and serves as an important catalytic cofactor in redox chemistry for proteins that carry out fundamental biological functions, important in growth and development. The deficit of copper can result in impaired energy production, abnormal glucose and cholesterol metabolism, increased oxidative damage, increased tissue iron (Fe) accrual, altered structure and function of circulating blood and immune cells, abnormal neuropeptides synthesis and processing, aberrant cardiac electrophysiology, impaired myocardial contractility, and persistent effects on the neurobehavioral and the immune system. Increased copper level has been found in several disorders like e.g.: Wilson's disease or Menke's disease. New findings with the great potential for impact in medicine include the use of copper-lowering therapy for antiangiogenesis, antifibrotic and anti-inflammatory purposes. The role of copper in formation of amyloid plaques in Alzheimer's disease, and successful treatment of this disorder in rodent model by copper chelating are also of interest. In this work we will try to describe essential aspects of copper in chosen diseases. We will represent the evidence available on adverse effect derived from copper deficiency and copper excess. We will try to review also the copper biomarkers (chosen enzymes) that help reflect the level of copper in the body.
铜（Cu）是一种存在于所有生物体内的必需微量元素，具有独特的能力，能够呈现两种不同的氧化还原状态——氧化态（Cu(2+)）和还原态（Cu(+)）。它是生存所必需的，并且作为氧化还原化学中的重要催化辅助因子，参与执行基本生物学功能的蛋白质的活动，对生长和发育至关重要。铜缺乏会导致能量产生受损、葡萄糖和胆固醇代谢异常、氧化损伤增加、组织铁（Fe）蓄积增加、循环血液和免疫细胞的结构与功能改变、神经肽合成与加工异常、心脏电生理异常、心肌收缩力受损，以及对神经行为和免疫系统产生持续影响。在诸如威尔逊病或门克斯病等多种疾病中已发现铜水平升高。在医学领域具有重大潜在影响的新发现包括使用降低铜水平的疗法来实现抗血管生成、抗纤维化和抗炎目的。铜在阿尔茨海默病淀粉样斑块形成中的作用，以及通过铜螯合在啮齿动物模型中成功治疗该疾病也备受关注。在这项工作中，我们将尝试描述所选疾病中铜的基本方面。我们将展示有关铜缺乏和铜过量产生的不良影响的现有证据。我们还将尝试综述有助于反映体内铜水平的铜生物标志物（所选酶）。
