A study of pathogenesis of neurodenerative disorders

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。主要结果如下。(1)我们制备了抗MG 160多肽和人trans-Golgi网络的多克隆抗体，两种抗体的染色模式与原始抗MG 160抗体几乎相似。我们现在正在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)In在16例ALS患者中，13.2%的贝茨细胞有高尔基体碎片，而在10例非ALS对照组中为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ALS痴呆的病理特征。(4)We披露，尸检的情况下，痴呆症与ALS并不罕见，在英国。(5)We报道了CSF中CSF tau、淀粉样蛋白β 1-40和淀粉样蛋白β 1 -42的组合测定（43）是阿尔茨海默病的生物学标志物。(6)我们分析了群马县13例脊髓小脑性共济失调2型患者。(7)We并分析了16例脊髓小脑共济失调6型患者。(8)我们报告了一个日本家族的肾上腺脑白质营养不良与三个碱基缺失（delGAG 29 l）的基因。(9)我们还报告了一个家族性脊髓小脑性共济失调，其临床特征slimar弗里德赖希共济失调。(10)我们报道了激活的小胶质细胞在铁蛋白的存在下引起铁依赖性脂质过氧化。

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.