ALTERATIONS IN THE LEVELS OF IRON, FERRITIN AND OTHER TRACE-METALS IN PARKINSONS-DISEASE AND OTHER NEURODEGENERATIVE DISEASES AFFECTING THE BASAL GANGLIA
ALTERATIONS IN THE LEVELS OF IRON, FERRITIN AND OTHER TRACE-METALS IN PARKINSONS-DISEASE AND OTHER NEURODEGENERATIVE DISEASES AFFECTING THE BASAL GANGLIA
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DOI：
10.1093/brain/114.4.1953
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发表时间：
1991-08-01
期刊：
影响因子：
14.5
通讯作者：
MARSDEN, CD
中科院分区：
文献类型：
--
作者：
DEXTER, DT;CARAYON, A;MARSDEN, CD
采用电感耦合等离子体光谱法测定了帕金森病（PD）、进行性核上性麻痹（PSP）、多系统萎缩伴纹状体黑质变性（MSA）和亨廷顿病（HD）患者冷冻死后脑组织中铁、铜、锌和锰的水平，并与对照组进行比较。 总铁水平被发现在基底神经节区升高，显示这些疾病的病理变化。 特别是，总铁含量增加，在黑质PD，PSP和MSA，但不是在HD。 在PSP，MSA和HD中，纹状体（壳核和/或尾状核）中的总铁水平增加，但在PD中不增加。 PD患者苍白球内总铁水平降低。 在研究的任何疾病中，基底神经节结构中的锰水平都没有一致的改变。 铜水平在PD的黑质中降低，在PSP的小脑中降低，在HD的壳核和可能的黑质中升高。 锌水平仅在PD中增加。在黑质和尾状核和外侧壳核中，使用放射免疫测定技术测量相同患者组中的铁结合蛋白铁蛋白的水平。 基底神经节铁水平升高通常与铁蛋白免疫反应性水平正常或升高相关，例如PSP和可能的MSA中的黑质，以及MSA中的壳核。 例外的是PD，其中有一个普遍的脑铁蛋白免疫反应性降低，甚至在substantia nigra.A总铁含量的增加似乎是一个反应，在一些运动障碍受影响的基底神经节区的神经变性。 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，锌浓度升高。 这些结果表明，在PD黑质铁处理的改变。 根据过量铁负荷存在于PD黑质中的形式，它可能有助于神经退行性过程。
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 radioimmunoassay 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.