铜蓝蛋白
神经退行性变
黑质
氧化应激
化学
炎症
神经毒性
去铁胺
去铁斯若
威尔逊病
疾病
帕金森病
生物化学
医学
内科学
毒性
有机化学
地中海贫血
作者
Petr Dušek,Per M. Roos,Tomasz Litwin,Susanne A. Schneider,Trond Peder Flaten,Jan Aaseth
标识
DOI:10.1016/j.jtemb.2014.05.007
摘要
Impaired cellular homeostasis of metals, particularly of Cu, Fe and Mn may trigger neurodegeneration through various mechanisms, notably induction of oxidative stress, promotion of α-synuclein aggregation and fibril formation, activation of microglial cells leading to inflammation and impaired production of metalloproteins. In this article we review available studies concerning Fe, Cu and Mn in Parkinson's disease and Wilson's disease. In Parkinson's disease local dysregulation of iron metabolism in the substantia nigra (SN) seems to be related to neurodegeneration with an increase in SN iron concentration, accompanied by decreased SN Cu and ceruloplasmin concentrations and increased free Cu concentrations and decreased ferroxidase activity in the cerebrospinal fluid. Available data in Wilson's disease suggest that substantial increases in CNS Cu concentrations persist for a long time during chelating treatment and that local accumulation of Fe in certain brain nuclei may occur during the course of the disease. Consequences for chelating treatment strategies are discussed.
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