Copper is an essential element in cells; it can act as either a recipient or a donor of electrons, participating in various reactions. However, an excess of copper ions in cells is detrimental as these copper ions can generate free radicals and increase oxidative stress. In multicellular organisms, copper metabolism involves uptake, distribution, sequestration, and excretion, at both the cellular and systemic levels. Mammalian enterocytes take in bioavailable copper ions from the diet in a Ctr1-dependent manner. After incorporation, cuprous ions are delivered to ATP7A, which pumps Cu⁺ from enterocytes into the blood. Copper ions arrive at the liver through the portal vein and are incorporated into hepatocytes by Ctr1. Then, Cu⁺ can be secreted into the bile or the blood via the Atox1/ATP7B/ceruloplasmin route. In the bloodstream, this micronutrient can reach peripheral tissues and is again incorporated by Ctr1. In peripheral tissue cells, cuprous ions are either sequestrated by molecules such as metallothioneins or targeted to utilization pathways by chaperons such as Atox1, Cox17, and CCS. Copper metabolism must be tightly controlled in order to achieve homeostasis and avoid disorders. A hereditary or acquired copper unbalance, including deficiency, overload, or misdistribution, may cause or aggravate certain diseases such as Menkes disease, Wilson disease, neurodegenerative diseases, anemia, metabolic syndrome, cardiovascular diseases, and cancer. A full understanding of copper metabolism and its roles in diseases underlies the identification of novel effective therapies for such diseases.

铜是细胞中必不可少的元素。它可以充当电子的接受者或供体，参与各种反应。然而，细胞中过量的铜离子是有害的，因为这些铜离子会产生自由基并增加氧化应激。在多细胞生物中，铜代谢涉及细胞和全身水平的摄取，分布，螯合和排泄。哺乳动物肠上皮细胞以依赖Ctr1的方式从饮食中摄取可生物利用的铜离子。掺入后，亚铜离子被输送到A​​TP7A，后者将Cu ⁺从肠细胞泵入血液。铜离子通过门静脉到达肝脏，并通​​过Ctr1整合到肝细胞中。然后，Cu ⁺可以通过Atox1 / ATP7B / ceruloplasmin途径分泌到胆汁或血液中。在血液中，这种微量营养素可以到达周围组织，并再次被Ctr1吸收。在外周组织细胞中，亚铜离子要么被诸如金属硫蛋白的分子螯合，要么被诸如Atox1，Cox17和CCS的分子伴侣靶向利用途径。铜代谢必须严格控制，以实现体内平衡和避免疾病。遗传性或后天性铜不平衡，包括缺乏，超负荷或分布不当，可能导致或加重某些疾病，例如Menkes病，Wilson病，神经退行性疾病，贫血，代谢综合征，心血管疾病和癌症。