The role of copper in neurodegenerative disease

被引:810
作者
Waggoner, DJ [1 ]
Bartnikas, TB [1 ]
Gitlin, JD [1 ]
机构
[1] Washington Univ, Sch Med, Edward Mallinckrodt Dept Pediat, St Louis, MO 63110 USA
关键词
copper; Menkes disease; Wilson disease; Aceruloplasminemia; amyotrophic lateral sclerosis; Alzheimer's disease; Prion disease;
D O I
10.1006/nbdi.1999.0250
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Copper is an essential trace metal which plays a fundamental role in the biochemistry of the human nervous system. Menkes disease and Wilson disease are inherited disorders of copper metabolism and the dramatic neurodegenerative phenotypes of these two diseases underscore the essential nature of copper in nervous system development as well as the toxicity of this metal when neuronal copper homeostasis is perturbed. Ceruloplasmin contains 95% of the copper found in human plasma and inherited loss of this essential ferroxidase is associated with progressive neurodegeneration of the retina and basal ganglia. Gain-of-function mutations in the cytosolic copper enzyme superoxide dismutase result in the motor neuron degeneration of amyotrophic lateral sclerosis and current evidence suggests a direct pathogenic role for copper in this process. Recent studies have also implicated copper in the pathogenesis of neuronal injury in Alzheimer's disease and the prion-mediated encephalopathies, suggesting that further elucidation of the mechanisms of copper trafficking and metabolism within the nervous system will he of direct relevance to our understanding of the pathophysiology and treatment of neurodegenerative disease. (C) 1999 Academic Press.
引用
收藏
页码:221 / 230
页数:10
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