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Research study on proteins participated with each stage of abnormal fibril formation in the neurons, glias and myofibers

参与神经元、胶质细胞和肌纤维异常原纤维形成各阶段的蛋白质研究

基本信息

批准号：
14570600
负责人：
KATAYAMA Sadao
金额：
$ 2.05万
依托单位：
HIROSHIMA UNIVERSITY
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
2002
资助国家：
日本
起止时间：
2002 至 2004
项目状态：
已结题

项目摘要

Progressive supranuclear palsy is a progressive degenerative disorder characterized by neuronal loss, gliosis and abnormal fibril formation of abnormally phosphorylated tau protein in neurons and glia cells, but the cause is not clear at present. For the purpose of clarifying the pathological significance of accumulation of tau protein in neurons and oligodendroglia in PSP, we morphologically classified NFT and coiled bodies (CB) in oligodendroglia in three PSP brains into four stages, using double staining for immunohistochemistry with AT8 antibody and modified Gallyas-Braak (GB) staining. AT8-positive neurons without abnormal fibril structure with GB staining were classified as stage I, AT8-positive neurons containing a few fibril structures with GB staining were classified stage II, AT8-positive neurons containing mature fibril structures were classified as stage III, and AT8 negative neurons containing abnormal fibril structures stained only with GB staining were classified as stage IV (ghost tangles). These stages were also assessed for CB. Then we counted the number of cells of each stage in various brain regions to investigate the relationship of NFT and CB with neuronal loss and gliosis. The results showed that there were very few stage IV NFT and CB, which reflect cell death, but that stage III NFT and CB were abundant. Moreover, CB were abundant in regions with severe neuronal loss. These results suggest that appearance of CB is closely associated with degenerative regions.

进行性核上性麻痹是一种进行性退行性疾病，其特征是神经元丢失、胶质增生以及神经元和胶质细胞中异常磷酸化tau蛋白的异常原纤维形成，但目前病因尚不清楚。为了阐明tau蛋白在PSP神经元和少突胶质细胞内聚集的病理学意义，我们采用免疫组化AT 8抗体和改良Gallyas-Braak（GB）双染法，将3例PSP脑内少突胶质细胞内的NFT和卷曲体（CB）形态学分为4期。将没有异常原纤维结构的AT 8阳性神经元用GB染色分类为I期，将含有少量原纤维结构的AT 8阳性神经元用GB染色分类为II期，将含有成熟原纤维结构的AT 8阳性神经元分类为III期，将含有仅用GB染色染色的异常原纤维结构的AT 8阴性神经元分类为IV期（鬼缠结）。还对CB的这些阶段进行了评估。然后对各期脑区的神经元数目进行计数，以探讨NFT和CB与神经元丢失和胶质增生的关系。结果表明，有非常少的IV期NFT和CB，反映细胞死亡，但III期NFT和CB是丰富的。此外，CB丰富的区域有严重的神经元损失。这些结果表明CB的出现与退行性变区域密切相关。