MRC Laboratory of Molecular Biology, Francis Crick Avenue, Cambridge Biomedical Campus, Cambridge CB2 0QH, U.K.
Biochem Soc Trans. 2021 Dec 17;49(6):2777-2786. doi: 10.1042/BST20210719.
Neurodegenerative diseases, such as Alzheimer's disease and Parkinson's disease, pose an increasingly severe burden for individuals and society in an ageing population. The causes and mechanisms of the diseases are poorly understood and as yet there are no effective treatments. Some of the molecular complexes involved in degeneration have been identified and electron microscopy has provided an essential tool in the investigations. The focus of this review is to show how electron microscopy has contributed historically to the understanding of disease and to summarize the most striking current advances. It does not seek to cover in detail the recent technical developments in microscopy, involving better microscopes, better electron detectors and more powerful image processing techniques, which have made possible the new insights. In many instances pathological filament assemblies are associated with brain cells that die in the disease, causing the observed symptoms such as dementia or movement disorders. Using electron microscopy it is now possible to go beyond morphological descriptions to produce atomic structures of many of the filaments. This information may help to understand the seeding and assembly of the filaments, with the aim of finding small molecule inhibitors that could potentially provide a form of treatment for the diseases.
神经退行性疾病,如阿尔茨海默病和帕金森病,在人口老龄化的背景下,给个人和社会带来了日益严重的负担。这些疾病的病因和机制还不甚清楚,目前也没有有效的治疗方法。已经确定了一些与退化有关的分子复合物,电子显微镜为研究提供了必不可少的工具。本篇综述的重点是展示电子显微镜在理解疾病方面的历史贡献,并总结当前最显著的进展。它并不试图详细介绍涉及更好的显微镜、更好的电子探测器和更强大的图像处理技术等方面的最新显微镜技术发展,这些发展使得新的见解成为可能。在许多情况下,病理性纤维组装与疾病中死亡的脑细胞有关,导致观察到的痴呆或运动障碍等症状。现在,使用电子显微镜可以超越形态描述,生成许多纤维的原子结构。这些信息可能有助于了解纤维的种子和组装,以期找到可能为这些疾病提供治疗形式的小分子抑制剂。