Immunocytochemical and ultrastructural studies of hyaline inclusions in sporadic motor neuron disease
Immunocytochemical and ultrastructural studies of hyaline inclusions in sporadic motor neuron disease
复制标题
散发性运动神经元病透明包涵体的免疫细胞化学和超微结构研究
作者：
S. Sasaki;S. Maruyama
摘要我们通过免疫细胞化学和超微结构研究了 6 例散发性运动神经元病 (MND) 病例的透明包涵体 (HI)。所有HI均含有大量泛素，部分HI在核心或中心被抗神经丝抗体染色，周围的光环未染色。没有 HI 被针对细胞骨架蛋白（例如高分子量微管相关蛋白和磷酸化 tau 蛋白）的抗体染色。从超微结构上看，HI 主要由直径约 20 nm 的细丝组成，比神经丝粗，并且含有细颗粒，并且通常包含四种特征轮廓中的一种或多种，即类似于布尼纳体的小电子致密材料、直径约 20 nm 的管状丝束、大电子致密核和随机排列的神经丝的焦点积累。透明包涵体可被视为散发性 MND 以及家族性肌萎缩侧索硬化症的特征性标志物之一。透明包涵体具有明显异质的超微结构，因此抗神经丝抗体的免疫反应性差异并不意外。
SummaryWe investigated hyaline inclusion bodies (HI) immunocytochemically and ultrastructurally in six cases of sporadic motor neuron disease (MND). All HI contained large amounts of ubiquitin and some HI were stained at the core or the center with anti-neurofilament antibody, with the surrounding halo unstained. No HI were stained with antibodies raised against cytoskeletal proteins such as high-molecular weight microtubule-associated proteins and phosphorylated tau. Ultrastructurally, HI were chiefly composed of filaments measuring about 20 nm in diameter thicker than neurofilaments, and contained fine granules and frequently one or more of four characteristic profiles, i.e., small electron-dense materials resembling Bunina bodies, bundles of tubular filaments measuring approximately 20 nm in diameter, large electron-dense cores, and focal accumulations of randomly arranged neurofilaments. Hyaline inclusions can be regarded as one of the characteristic markers for sporadic MND as well as familial amyotrophic lateral sclerosis. Hyaline inclusions have a markedly heterogeneous ultrastructure and, therefore, differences in immunoreactivity with antineurofilament antibodies are not unexpected.
运动神经元疾病患者和对照患者轴突肿胀中的磷酸依赖性和独立神经丝表位。
DOI：
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发表时间：
1987
期刊：
Laboratory investigation; a journal of technical methods and pathology
影响因子：
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作者：
Schmidt,ML;Carden,MJ;Lee,VM;Trojanowski,JQ
通讯作者：
Trojanowski,JQ