Iron chelation in the treatment of neurodegenerative diseases
Iron chelation in the treatment of neurodegenerative diseases
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DOI：
10.1016/j.jtemb.2016.03.010
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发表时间：
2016-01-01
影响因子：
3.5
通讯作者：
Aaseth, Jan
中科院分区：
文献类型：
--
作者：
Dusek, Petr;Schneider, Susanne A.;Aaseth, Jan
脑铁调节紊乱在神经退行性疾病中几乎普遍存在。越来越多的证据表明，铁沉积物的增加可能会导致退行性变化。因此，铁螯合疗法在许多神经系统疾病中的效果已得到研究，包括罕见的遗传综合征（伴有脑铁积累的神经变性）以及常见的散发性疾病，如帕金森病、阿尔茨海默病和多发性硬化症。这篇综述总结了了解铁在神经退行性疾病病因学中的作用的最新进展。表格中系统地列出了调查铁螯合疗法对神经退行性疾病的影响的研究结果。铁螯合剂，特别是跨越血脑屏障的化合物去铁酮，能够减少 MRI 记录的异常高浓度区域的脑铁。然而，目前还没有令人信服的证据表明除铁疗法对任何神经系统疾病有临床效果。然而，一些研究表明，它可以预防或减缓多种疾病的进展，例如铜蓝蛋白血症、泛酸激酶相关的神经变性或帕金森病。 (C) 2016 爱思唯尔有限公司。版权所有。
Disturbance of cerebral iron regulation is almost universal in neurodegenerative disorders. There is a growing body of evidence that increased iron deposits may contribute to degenerative changes. Thus, the effect of iron chelation therapy has been investigated in many neurological disorders including rare genetic syndromes with neurodegeneration with brain iron accumulation as well as common sporadic disorders such as Parkinson's disease, Alzheimer's disease, and multiple sclerosis. This review summarizes recent advances in understanding the role of iron in the etiology of neurodegeneration. Outcomes of studies investigating the effect of iron chelation therapy in neurodegenerative disorders are systematically presented in tables. Iron chelators, particularly the blood brain barrier-crossing compound deferiprone, are capable of decreasing cerebral iron in areas with abnormally high concentrations as documented by MRI. Yet, currently, there is no compelling evidence of the clinical effect of iron removal therapy on any neurological disorder. However, several studies indicate that it may prevent or slow down disease progression of several disorders such as aceruloplasminemia, pantothenate kinase-associated neurodegeneration or Parkinson's disease. (C) 2016 Elsevier GmbH. All rights reserved.