铜缺乏
门克斯病
线粒体
铜
细胞色素c氧化酶
生物
ATP7A型
背景(考古学)
铜代谢
细胞生物学
生物化学
化学
运输机
基因
有机化学
古生物学
作者
Natalie M. Garza,Abhinav B. Swaminathan,Krishna Prahlad Maremanda,Mohammad Zulkifli,Vishal M. Gohil
标识
DOI:10.1016/j.tem.2022.11.001
摘要
Copper is an essential micronutrient that serves as a cofactor for enzymes involved in diverse physiological processes, including mitochondrial energy generation. Copper enters cells through a dedicated copper transporter and is distributed to intracellular cuproenzymes by copper chaperones. Mitochondria are critical copper-utilizing organelles that harbor an essential cuproenzyme cytochrome c oxidase, which powers energy production. Mutations in copper transporters and chaperones that perturb mitochondrial copper homeostasis result in fatal genetic disorders. Recent studies have uncovered the therapeutic potential of elesclomol, a copper ionophore, for the treatment of copper deficiency disorders such as Menkes disease. Here we review the role of copper in mitochondrial energy metabolism in the context of human diseases and highlight the recent developments in copper therapeutics.
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