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A study of pathogenesis of neurodenerative disorders

神经退行性疾病发病机制的研究

基本信息

批准号：
09470152
负责人：
OKAMOTO Koichi
金额：
$ 8.26万
依托单位：
Gunma University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (B)
财政年份：
1997
资助国家：
日本
起止时间：
1997 至 1998
项目状态：
已结题

项目摘要

We did the morphological, immnocytochemical and molecular researches for amyotrophic lateral scleosis (ALS) spinocerebellar ataxia and Alzheimer's disease. The main results are follows. (1) We made polyclonal antibodies against peptides of MG 160 and human trans-Golgi network, and staining pattern of both antibodies were almost similar to original anti-MG 160 antibody. We are now doing immunohistochemical study of Golgi appatatus in the neurons of many neurodegenerative disordes using these antibodies. (2)In 16 ALS patients, 13.2% of counted Betz cells had fragmented Golgi apparatus in contrast to 0.6% in the 10 non-ALS controls. (3)The temporal lobes of 30 clinically and pathologically confirmed patients with ALS were examined, and we disclosed the characteristic pathology of dementia with ALS.(4)We disclosed that autopsied cases of dementia with ALS were not rarely present in UK.(5)We reported that combination assay for CSF tau, amyloid beta 1-40 and amyloid beta1-42(43) in CSF is a biological marker of Alzheimer's disease. (6) We analysed 13 patients with spinocerebellar ataxia type 2 in Gunma prefecture. (7)We also analysed 16 spinocerebellar ataxia type 6 patients. (8) We reported a Japanese family with adrenoleukodystrophy with a three base deletion (delGAG29l) in the gene. (9) We also reported a familial spinocerebellar ataxia, which has clinical featutes slimar to Friedreich's ataxia. (10) We reported that activated microglia cause iron-dependent lipid peroxidation in the presence of ferritin.

我们对肌萎缩侧索硬化症(ALS)、脊髓小脑性共济失调和阿尔茨海默病进行了形态学、免疫细胞化学和分子生物学研究。主要研究结果如下。(1)制备了抗人MG 160多肽和人反式高尔基网络的多克隆抗体，两种抗体的染色图谱与原抗体基本一致。我们现在正在使用这些抗体对许多神经退行性疾病的神经元中的高尔基体进行免疫组织化学研究。(2)16例ALS患者计数的Betz细胞高尔基体碎片率为13.2%，10例非ALS患者为0.6%。(3)对30例经临床和病理证实的ALS患者的颞叶进行了检查，揭示了痴呆合并ALS的特征性病理。(4)我们揭示了痴呆合并ALS的尸检病例在英国并不少见。(5)我们报道了脑脊液tau、淀粉样β1-40和淀粉样β1-42(43)的联合检测是阿尔茨海默病的生物学标志。(6)对群马州13例脊髓小脑性共济失调2型患者进行分析。(7)对16例脊髓小脑性共济失调6型患者进行分析。(8)我们报道了一个日本肾上腺脑白质营养不良家系，该基因有三个碱基缺失(DelGAG291)。(9)我们还报告了一例家族性脊髓小脑性共济失调，其临床特征与Friedreich‘s共济失调相似。(10)我们报道了在铁蛋白存在的情况下，激活的小胶质细胞引起铁依赖的脂质过氧化。