Role of copper in human neurological disorders
Role of copper in human neurological disorders
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DOI：
10.1093/ajcn/88.3.855s
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发表时间：
2008-09-01
影响因子：
7.1
通讯作者：
Kaler, Stephen G.
中科院分区：
文献类型：
--
作者：
Desai, Vishal;Kaler, Stephen G.
铜是存在于所有组织中的微量元素，并且是细胞呼吸、肽酰胺化、神经递质生物合成、色素形成和结缔组织强度所必需的。铜是许多酶的辅助因子，在中枢神经系统发育中起着重要作用;低浓度的铜可能导致发育不完全，而过量的铜可能有害。铜可能通过Haber-Weiss反应参与自由基的产生，导致线粒体损伤、DNA断裂和神经元损伤。在许多人类神经系统疾病中异常铜转运和异常铜-蛋白质相互作用的证据支持这种痕量金属对适当的神经发育和神经功能的至关重要性。涉及铜稳态的人类疾病的生化表型表明可能适用于一般人群的铜状态的生物标志物。
Copper is a trace element present in all tissues and is required for cellular respiration, peptide amidation, neurotransmitter biosynthesis, pigment formation, and connective tissue strength. Copper is a cofactor for numerous enzymes and plays an important role in central nervous system development; low concentrations of copper may result in incomplete development, whereas excess copper maybe injurious. Copper may be involved in free radical production, via the Haber-Weiss reaction, that results in mitochondrial damage, DNA breakage, and neuronal injury. Evidence of abnormal copper transport and aberrant copper-protein interactions in numerous human neurological disorders supports the critical importance of this trace metal for proper neurodevelopment and neurological function. The biochemical phenotypes of human disorders that involve copper homeostasis suggest possible biomarkers of copper status that may be applicable to general populations.