Dexter D T, Carayon A, Javoy-Agid F, Agid Y, Wells F R, Daniel S E, Lees A J, Jenner P, Marsden C D
Parkinson's Disease Society Research Centre, University Department of Neurology, London, UK.
Brain. 1991 Aug;114 ( Pt 4):1953-75. doi: 10.1093/brain/114.4.1953.
Levels of iron, copper, zinc and manganese were measured by inductively coupled plasma spectroscopy in frozen postmortem brain tissue from patients with Parkinson's disease (PD), progressive supranuclear palsy (PSP), multiple system atrophy with strionigral degeneration (MSA), and Huntington's disease (HD) compared with control subjects. Total iron levels were found to be elevated in the areas of basal ganglia showing pathological change in these disorders. In particular, total iron content was increased in substantia nigra in PD, PSP and MSA, but not in HD. Total iron levels in the striatum (putamen and/or caudate nucleus) were increased in PSP, MSA and HD but not in PD. Total iron levels were decreased in the globus pallidus in PD. There were no consistent alterations of manganese levels in basal ganglia structures in any of the diseases studied. Copper levels were decreased in the substantia nigra in PD, and in the cerebellum in PSP, and were elevated in the putamen and possibly substantia nigra in HD. Zinc levels were only increased in PD, in substantia nigra and in caudate nucleus and lateral putamen. Levels of the iron binding protein ferritin were measured in the same patient groups using a radio-immunoassay technique. Increased iron levels in basal ganglia were generally associated with normal or elevated levels of ferritin immunoreactivity, for example, the substantia nigra in PSP and possibly MSA, and in putamen in MSA. The exception was PD where there was a generalized reduction in brain ferritin immunoreactivity, even in the substantia nigra. An increase in total iron content appears to be a response to neurodegeneration in affected basal ganglia regions in a number of movement disorders. However, only in PD was there an increased total iron level, decreased ferritin content, decreased copper content, and an increased zinc concentration in substantia nigra. These findings suggest an alteration of iron handling in the substantia nigra in PD. Depending on the form in which the excess iron load exists in nigra in PD, it may contribute to the neurodegenerative process.
采用电感耦合等离子体光谱法，对帕金森病（PD）、进行性核上性麻痹（PSP）、纹状体黑质变性型多系统萎缩（MSA）和亨廷顿病（HD）患者死后冷冻脑组织中的铁、铜、锌和锰水平进行了测量，并与对照组受试者进行了比较。发现在这些疾病中出现病理改变的基底神经节区域，总铁水平升高。特别是，PD、PSP和MSA患者的黑质总铁含量增加，而HD患者则未增加。PSP、MSA和HD患者纹状体（壳核和/或尾状核）的总铁水平升高，而PD患者则未升高。PD患者苍白球的总铁水平降低。在所研究的任何一种疾病中，基底神经节结构中的锰水平均无一致变化。PD患者黑质中的铜水平降低，PSP患者小脑中的铜水平降低，而HD患者壳核及可能的黑质中的铜水平升高。锌水平仅在PD患者的黑质、尾状核和外侧壳核中升高。使用放射免疫测定技术对同一患者组的铁结合蛋白铁蛋白水平进行了测量。基底神经节中铁水平升高通常与铁蛋白免疫反应性正常或升高有关，例如，PSP和可能的MSA患者的黑质，以及MSA患者的壳核。例外情况是PD，即使在黑质中，脑铁蛋白免疫反应性也普遍降低。在一些运动障碍中，受影响的基底神经节区域总铁含量增加似乎是对神经退行性变的一种反应。然而，只有在PD中，黑质中的总铁水平升高、铁蛋白含量降低、铜含量降低以及锌浓度升高。这些发现表明PD患者黑质中铁处理存在改变。根据PD患者黑质中过量铁负荷的存在形式，它可能会促成神经退行性变过程。
