King M E, Ghoshal N, Wall J S, Binder L I, Ksiezak-Reding H
Department of Cell and Molecular Biology, Northwestern University Medical School, 303 E. Chicago Ave., Chicago, IL 60611, USA.
Am J Pathol. 2001 Apr;158(4):1481-90. doi: 10.1016/S0002-9440(10)64099-0.
Pick's and Alzheimer's diseases are distinct neurodegenerative disorders both characterized in part by the presence of intracellular filamentous tau protein inclusions. The tight bundles of paired helical filaments (PHFs) of tau protein found in Alzheimer's disease (AD) differ from the tau filaments of Pick's disease in their morphology, distribution, and pathological structure as identified by silver impregnation. The filaments of Pick's disease are loosely arranged in pathognomonic spherical inclusions found in ballooned neurons, whereas the tau pathology of AD is classically described as a triad of neuropil threads, neurofibrillary tangles, and dystrophic neurites surrounding and invading plaques. In this study we used the high-resolution technique of scanning transmission electron microscopy to characterize and compare the filaments found in Pick's disease with those found in AD. In addition, we determined the mass/nm length and density of arachidonic acid-induced in vitro-assembled filaments. Three morphologically distinct populations of Pick's filaments were identified but each was indistinguishable from AD-PHFs in mass/nm length and density. Filaments assembled in vitro from single isoforms were similar in mass/nm length, but less dense than AD-PHFs and Pick's disease filaments. Finally, we provide clear structural evidence that a PHF, whether found in disease or assembled in vitro, is composed of two distinct intertwined filaments.
皮克病和阿尔茨海默病是不同的神经退行性疾病,二者的部分特征均为细胞内存在丝状tau蛋白包涵体。在阿尔茨海默病(AD)中发现的tau蛋白双螺旋丝(PHF)紧密束,在形态、分布和病理结构上与皮克病的tau丝不同,这是通过银浸染鉴定的。皮克病的丝松散地排列在气球样神经元中发现的特征性球形包涵体内,而AD的tau病理经典地描述为神经毡丝、神经原纤维缠结以及围绕和侵入斑块的营养不良性神经突三联征。在本研究中,我们使用扫描透射电子显微镜的高分辨率技术来表征和比较皮克病中发现的丝与AD中发现的丝。此外,我们确定了花生四烯酸诱导的体外组装丝的质量/纳米长度和密度。鉴定出了皮克丝的三个形态学上不同的群体,但每个群体在质量/纳米长度和密度上与AD-PHFs没有区别。由单一异构体体外组装的丝在质量/纳米长度上相似,但密度低于AD-PHFs和皮克病丝。最后,我们提供了明确的结构证据,即PHF,无论在疾病中发现还是在体外组装,都是由两条不同的交织丝组成。