Apolipoprotein D in the Niemann-Pick type C disease mouse brain: an ultrastructural immunocytochemical analysis.
Apolipoprotein D in the Niemann-Pick type C disease mouse brain: an ultrastructural immunocytochemical analysis.
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Niemann-Pick C 型疾病小鼠大脑中的载脂蛋白 D：超微结构免疫细胞化学分析。
DOI：
10.1023/a:1023993405851
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发表时间：
2002
期刊：
影响因子：
--
通讯作者：
Patel,ShutishC
中科院分区：
文献类型：
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作者：
Ong,Wei-Yi;Hu,Chang-Yong;Patel,ShutishC
增强的apoD基因表达和异常高水平的apoD蛋白在脑中的积累先前已被证明是神经退行性疾病尼曼-匹克C型疾病（NP-C）的特征。在本研究中，我们使用免疫细胞化学和光学和电子显微镜来阐明Balb/c NIHnpc 1 −/−小鼠脑中apoD的细胞和亚细胞分布。正常小鼠大脑表现出低水平的apoD表达胶质细胞，特别是在小脑白色物质中。与此相反，丰富的，强烈apoD-免疫标记的细胞中观察到选定的灰质核，包括苍白球，丘脑，黑质，并在白色物质束内囊和小脑的NP-C小鼠大脑。这些大脑区域先前已被证明在NP-C小鼠中显示出最显著的神经退行性变化。超微结构分析显示，密集的apoD免疫反应的细胞，具有少突胶质细胞的形态特征，星形胶质细胞和轻染色的核膜。这些结果定义的细胞和亚细胞模式的apoD在NP-C小鼠脑的表达，并建议这种脂质运载蛋白在这种疾病的病理生理可能的作用。
Enhanced apoD gene expression and abnormally high levels of apoD protein accumulation in the brain have been previously documented as features of the neurodegenerative disorder, Niemann-Pick Type C disease (NP-C). In the present study we have used immunocytochemistry and light and electron microscopy to elucidate the cellular and subcellular distribution of apoD in the Balb/c NIHnpc1−/−mouse brain. The normal mouse brain demonstrates low levels of apoD-expressing glia particularly in the cerebellar white matter. In contrast, abundant, strongly apoD-immunolabeled cells were observed in select grey matter nuclei, including the globus pallidus, thalamus, and substantia nigra, and in white matter tracts within the internal capsule and cerebellum of NP-C mouse brain. These brains regions have been previously shown to display the most significant neurodegenerative changes in the NP-C mouse. Ultrastructural analysis revealed dense apoD immunoreactivity on the nuclear envelopes of cells that have the morphological features of oligodendrocyte precursor-like cells and light staining on astrocytes. These results define the cellular and subcellular pattern of expression of apoD in NP-C mouse brain and suggest a possible role for this lipocalin in the pathophysiology of this disorder.