Astrocytic deformity and globular structures are characteristic of the brains of patients with aceruloplasminemia
Astrocytic deformity and globular structures are characteristic of the brains of patients with aceruloplasminemia
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DOI：
10.1093/jnen/61.12.1069
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发表时间：
2002-12-01
影响因子：
3.2
通讯作者：
Ikeda, SI
中科院分区：
文献类型：
--
作者：
Kaneko, K;Yoshida, K;Ikeda, SI
血浆无铜蓝蛋白血症是一种有趣的疾病，其研究有助于阐明铁诱导的氧化应激如何参与神经元细胞死亡。为了研究与氧化应激相关的神经病理学特征，我们对5例浆细胞蛋白血症患者的大脑进行了组织病理学和免疫组织化学检查。所有患者的病理结果基本相似。在额叶皮层;铁沉积和神经元细胞损失是微不足道的，但在基底神经节（特别是在尾状核和壳核）中;注意到严重的铁过载和广泛的神经元损失。铁沉积更突出的星形胶质细胞比在这两个地区的神经元。4-羟基壬烯醛（HNE）是最具生理活性的脂质过氧化物之一，通过免疫染色在神经元和星形胶质细胞上强烈检测到。在纹状体中观察到显著变形的星形胶质细胞。这些星形胶质细胞类似于阿尔茨海默氏症1型星形胶质细胞。球状结构被认为是成比例的铁沉积的程度。它们清楚地与抗胶质细胞酸性蛋白（GFAP）和抗S-100抗体反应，并含有胶质纤维样细丝，但对神经元标记蛋白（如神经丝和突触素）的抗体没有或只有微弱的免疫反应性。因此，球状结构可能起源于星形胶质细胞。该结构还与抗HNE和抗泛素抗体反应呈阳性。我们的结论是，星形胶质细胞畸形和球状结构的特征性神经病理学特征的浆细胞蛋白血症，并密切相关的铁超载和随后的氧化应激。
Aceruloplasminemia is an interesting disease, the study of which helps elucidate how iron-induced oxidative stress is involved in neuronal cell death. In order to study the neuropathological characteristics associated with oxidative stress, we scrutinized the brains of 5 patients with aceruloplasminemia histopathologically and immunohistochemically. The pathological findings were essentially similar in all patients. In the frontal cortices; iron deposition and neuronal cell loss were trivial, but in the basal ganglia (especially in the caudate nucleus and putamen); severe iron overload and extensive neuronal loss were noted. Iron deposition was more prominent in the astrocytes than in the neurons in both regions. 4-hydroxynonenal (HNE), one of the most physiologically active lipid peroxides, was strongly detected on neurons and astrocytes by immunostaining. Markedly deformed astrocytes were observed in the striatum. These astrocytes were similar to Alzheimer type 1 astrocytes. Globular structures were seen in proportion to the degree of iron deposition. They clearly reacted with anti-glial fibrillary acidic protein (GFAP) and anti-S-100 antibodies and contained glial fibril-like filaments, but showed no or only faint immunoreactivity to antibodies for neuronal marker proteins, such as neurofilament and synaptophysin. Therefore, the globular structures presumably originated from astrocytes. The structures also reacted positively to anti-HNE and anti-ubiquitin antibodies. We conclude that astrocytic deformities and globular structures are characteristic neuropathological features of aceruloplasminemia and are closely linked to iron overload and subsequent oxidative stress.