Iron and copper in mitochondrial diseases.

Iron and copper in mitochondrial diseases.
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DOI:
10.1016/j.cmet.2013.02.004
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发表时间:
2013-03-05
期刊:
影响因子:
29
通讯作者:
Andrews NC
Andrews NC
中科院分区:
生物学1区
文献类型:
--
作者:
Xu W;Barrientos T;Andrews NC

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过渡金属经常用作酶和携氧蛋白质的辅助因子,利用它们获得和失去单个电子的倾向。金属在线粒体中尤其重要,它们在 ATP 的产生和活性氧的解毒中发挥着重要作用。同时,过渡金属(特别是铁和铜)会促进有害自由基的形成,因此需要仔细控制金属浓度和亚细胞区室化。我们总结了目前对哺乳动物线粒体生物学中铁和铜的理解,并讨论了与线粒体金属稳态失常相关的人类疾病。
Transition metals are frequently used as co-factors for enzymes and oxygen-carrying proteins that take advantage of their propensity to gain and lose single electrons. Metals are particularly important in mitochondria, where they play essential roles in the production of ATP and detoxification of reactive oxygen species. At the same time, transition metals (particularly Fe and Cu) can promote the formation of harmful radicals, necessitating meticulous control of metal concentration and subcellular compartmentalization. We summarize our current understanding of Fe and Cu in mammalian mitochondrial biology, and discuss human diseases associated with aberrations in mitochondrial metal homeostasis.
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