Alterations in levels of iron, ferritin, and other trace metals in neurodegenerative diseases affecting the basal ganglia. The Royal Kings and Queens Parkinson's Disease Research Group.

Alterations in levels of iron, ferritin, and other trace metals in neurodegenerative diseases affecting the basal ganglia. The Royal Kings and Queens Parkinson's Disease Research Group.
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DOI:
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发表时间:
1992
影响因子:
11.2
通讯作者:
D. Dexter;P. Jenner;A. Schapira;C. Marsden
D. Dexter;P. Jenner;A. Schapira;C. Marsden
中科院分区:
医学1区
文献类型:
--
作者:
D. Dexter;P. Jenner;A. Schapira;C. Marsden

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以前我们已经证明,帕金森病(PD)黑质(SN)的细胞死亡与铁含量增加但铁结合蛋白铁蛋白水平降低有关。其他金属离子水平的变化也被观察到;铜水平下降,而锌水平上升。铁、铁蛋白和其他金属离子的这些变化在帕金森病的病理生理学中的重要性取决于它们是否是疾病特有的。我们检测了进行性核上性瘫痪(PSP)、多系统萎缩(MSA)(表现为黑质和纹状体的病理改变)和亨廷顿病(HD)(表现为纹状体的病理改变)患者死后组织中铁、铜、锌、锰和铁蛋白的水平。在这些疾病中,基底节区域的总铁水平升高,显示出病理变化。特别是,PD、PSP和MSA的SN中全铁含量增加,而HD中的全铁含量没有增加。PSP组、MSA组和HD组纹状体(尾状核和/或壳核)总铁水平升高,而PD组无明显变化。在所研究的任何疾病中,基底节中的锰水平都没有持续的变化。PD组黑质和PSP组小脑铜含量降低,壳核和HD组黑质铜含量升高。只有黑质、尾状核和壳核中的锌含量在帕金森氏核中升高。
Previously we have shown that cell death in the substantia nigra (SN) in Parkinson's disease (PD) is associated with an increase in iron content but a decrease in the level of the iron-binding protein ferritin. Alterations in other metal ion levels were also observed; copper levels were reduced, whereas zinc levels were increased. The importance of these changes in iron, ferritin, and other metal ions in the pathophysiology of PD depends on whether they are specific to the illness. We measured levels of iron, copper, zinc, manganese, and ferritin in postmortem tissue from patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) (which shows pathology in the SN and striatum) and Huntington's disease (HD) (which shows pathological changes in the striatum, compared with control subjects). Total iron levels were elevated in areas of the basal ganglia showing pathological changes in these disorders. In particular, total iron content was increased in SN in PD, PSP, and MSA, but not in HD. Total iron levels in the striatum (caudate nucleus and/or putamen) were increased in PSP, MSA, and HD, but not in PD. There were no consistent alterations in manganese levels in the basal ganglia in any of the diseases studied. Copper levels were decreased in the SN in PD and in the cerebellum in PSP, and were elevated in the putamen and possibly the SN in HD. Zinc levels were only increased in PD in the SN, the caudate nucleus, and the putamen.(ABSTRACT TRUNCATED AT 250 WORDS)