Department of Pathology and Molecular Medicine, Third Faculty of Medicine, Charles University and Thomayer Hospital, 100 00 Prague, Czech Republic.
Department of Pathology, First Faculty of Medicine, Charles University, and General University Hospital, 100 00 Prague, Czech Republic.
Int J Mol Sci. 2020 Dec 22;22(1):7. doi: 10.3390/ijms22010007.
Neurodegenerative diseases are characterized by the deposition of specific protein aggregates, both intracellularly and/or extracellularly, depending on the type of disease. The extracellular occurrence of tridimensional structures formed by amyloidogenic proteins defines Alzheimer's disease, in which plaques are composed of amyloid β-protein, while in prionoses, the same term "amyloid" refers to the amyloid prion protein. In this review, we focused on providing a detailed didactic description and differentiation of diffuse, neuritic, and burnt-out plaques found in Alzheimer's disease and kuru-like, florid, multicentric, and neuritic plaques in human transmissible spongiform encephalopathies, followed by a systematic classification of the morphological similarities and differences between the extracellular amyloid deposits in these disorders. Both conditions are accompanied by the extracellular deposits that share certain signs, including neuritic degeneration, suggesting a particular role for amyloid protein toxicity.
神经退行性疾病的特征是特定蛋白质聚集物的沉积，无论是细胞内还是细胞外，具体取决于疾病的类型。由淀粉样蛋白形成的三维结构在细胞外的出现定义了阿尔茨海默病，其中斑块由淀粉样 β 蛋白组成，而在朊病毒病中，相同的术语“淀粉样”指的是淀粉样朊病毒蛋白。在这篇综述中，我们重点详细介绍了在阿尔茨海默病中发现的弥漫性、神经突性和耗竭性斑块，以及人类传染性海绵状脑病中的库鲁样、华丽型、多中心和神经突性斑块之间的差异，并对这些疾病中外泌体淀粉样沉积物的形态相似性和差异进行了系统分类。这两种情况都伴随着具有某些特征的细胞外沉积物，包括神经突变性，这表明淀粉样蛋白毒性具有特定作用。
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