Neuropathological research of argyrophilic grain dementia

嗜银性颗粒性痴呆的神经病理学研究

• 批准号: 16500230
• 负责人: YOSHIDA Mari
• 金额: $ 1.79万
• 依托单位: Aichi Medical University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2004
• 资助国家: 日本
• 起止时间: 2004 至 2006
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-16500230/
• 关键词: argyrophilic grain; tauopathy; argyrpohilic grain dementia; Alzheimer's neurofibrillary tangles; pretangle; coiled body; non-Alzheimer type dementia; tau isoform; argyrophilic grain; 辺縁系; タウ アイソフォーム; 非アルツハイマー型痴呆

Argyrophilic grain dementia (AGD) is a late onset tauopathy characterized by the occurrence of argyrophilic and tau-immunoreactive grains most abundant in the limbic system, especially in the CA1 region of the hippocampus, the entorhinal and transentorhinal cortex, the adjacent temporal isocortex, and the amygdala. These oval, spindle-shaped or comma-like grains can be best detected by the Gallyas-Braak silver technique. Many tau-immunoreactive pretangles with poor neurofibrillary tangles (NFT)-formation are distributed in the limbic system. Tau-immunoreactive glial changes seen in oligodendroglial coiled bodies and astrocytes represent a consistent finding. Ballooned neurons expressing a B-crystallin are constant features in the amygdala of AGD brains. NFT found in AGD patients generally correspond to early (transitional and limbic) Braak stages.The prevalence of AGD increases with advancing age. Frequency of AGD in 32 centenarians among 3328 autopsied brains was 31.3%. In the demented group, 9 (39.1%) of 23 Cases were found with AGD, while in the non-demented group, AGs were found on only 1 (11.1%) of nine cases. Dementia caused by pure AGD accounted for 13% (3/23) among demented subjects. Frequency of AGD in 110 cases with amyotrophic lateral sclerosis (age 32〜86) was 20%. Clinically 7 cases showed mild mental symptoms. Clinicopathological spectrum of AGD remains to be unclear.We reported a case with severe frontotemporal atrophy accompanied by numerous AGs. At the age of 49, he exhibited personality change, speech disorder and abnormal behavior. The neuropathological findings of extensive-type AGD would constitute a part of pathological background of frontotemporal dementia.AGs were immunopositive for 4-repeat tau on immunohistochemical study, using two monoclonal antibodies, RD3, RD4 that effectively distinguish between 3-repeat and 4-repeat tau.

嗜银颗粒痴呆（AGD）是一种迟发性tau蛋白病，其特征在于嗜银和tau蛋白免疫反应颗粒在边缘系统中最丰富的发生，特别是在海马的CA 1区、内嗅和经内嗅皮质、邻近的颞等皮质和杏仁核中。这些椭圆形、纺锤形或逗号状颗粒最好用Gallyas-Braak银技术检测。边缘系统中分布有许多tau蛋白免疫反应阳性的神经元缠结（NFT）形成较差的前角。在少突胶质细胞卷曲体和星形胶质细胞中观察到的Tau免疫反应性胶质细胞变化代表了一致的发现。表达B-晶体蛋白的神经元是AGD大脑杏仁核中的恒定特征。在AGD患者中发现的NFT通常对应于早期（过渡期和边缘）Braak分期。AGD的患病率随着年龄的增长而增加。在3328例百岁老人尸检中，32例出现AGD，占31.3%。痴呆组23例中9例（39.1%）有AGD，非痴呆组9例中1例（11.1%）有AGD。单纯AGD引起的痴呆占13%（3/23）。110例肌萎缩侧索硬化患者（年龄32 ~ 86岁）AGD发生率为20%。临床表现为轻度精神症状者7例。我们报告一个严重的额颞叶萎缩并伴有大量AGs的病例。49岁时，他表现出人格改变，言语障碍和行为异常。广泛型AGD的神经病理学表现可构成额颞叶痴呆的病理背景之一，免疫组化染色显示AGs中4-重复tau蛋白阳性，RD 3、RD 4单克隆抗体可有效区分3-重复和4-重复tau蛋白。

项目成果

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degneration and amyotrophic lateral sclerosis

TDP-43 是额颞叶变性和肌萎缩侧索硬化症中泛素阳性 tau 阴性包涵体的组成部分

• 发表时间: 2006
• 期刊: Biochemical and Biophysical Research Communications 351
• 作者: Arai T;Hasegawa M;Akiyama H;Ikeda K;Nonaka T;Mori H;Mann D;Tsuchiya K;Yoshida M;Hashizume Y;Oda T
• 通讯作者: Oda T

Distribution of tuft-shaped astrocytes in the cerebral cortex in progressive supranuclear palsy

进行性核上性麻痹大脑皮层簇状星形胶质细胞的分布

• 发表时间: 2004
• 期刊: Acta Neuropathol 108
• 作者: Iwasaki Y;Yoshida M;Hattori M;Goto A;Aiba I;Hashizume Y;Sobue G
• 通讯作者: Sobue G

Immunohistochemical study of tau isoforms in sporadic tauopathies

散发性 tau 病中 tau 亚型的免疫组织化学研究

• 发表时间: 2005
• 期刊: Dementia Japan 19-3
• 作者: Yoshida M

Argyrophilic grain dementiaの神経病理

嗜银性颗粒性痴呆的神经病理学

• 发表时间: 2004
• 期刊: 神経進歩 48・3
• 作者: Uchida;Y.;Ohshima;T.;Sasaki;Y.;Suzuki;H.;Yanai;S.;Yamashita;N.;Nakamura;F.;Takei;K.;Ihara;Y.;Mikoshiba;K.;Kolattukudy;P.;Honnorat;J.;Goshima;Y.;吉田 眞理
• 通讯作者: 吉田 眞理

孤発性タウオパチーのタウアイソフォームの免疫組織学的検討

散发性 tau 病中 tau 亚型的免疫组织学研究

• 发表时间: 2005
• 期刊: Dementia Japan 19・3
• 作者: Ohnishi;H. et al.;Guan J.L.;吉田 眞理
• 通讯作者: 吉田 眞理