Department of Chemistry , University of Cambridge , Lensfield Road , Cambridge CB2 1EW , United Kingdom.
MRC Laboratory of Molecular Biology , Francis Crick Avenue , Cambridge CB2 0QH , United Kingdom.
ACS Chem Neurosci. 2018 Jun 20;9(6):1276-1282. doi: 10.1021/acschemneuro.8b00094. Epub 2018 Apr 8.
The ordered assembly of amyloidogenic proteins causes a wide spectrum of common neurodegenerative diseases, including Alzheimer's and Parkinson's diseases. These diseases share common features with prion diseases, in which misfolded proteins can self-replicate and transmit disease across different hosts. Deciphering the molecular mechanisms that underlie the amplification of aggregates is fundamental for understanding how pathological deposits can spread through the brain and drive disease. Here, we used single-molecule microscopy to study the assembly and replication of tau at the single aggregate level. We found that tau aggregates have an intrinsic ability to amplify by filament fragmentation, and determined the doubling times for this replication process by kinetic modeling. We then simulated the spreading time for aggregates through the brain and found this to be in good agreement with both the observed time frame for spreading of pathological tau deposits in Alzheimer's disease and in experimental models of tauopathies. With this work we begin to understand the physical parameters that govern the spreading rates of tau and other amyloids through the human brain.
淀粉样蛋白的有序组装导致了广泛的常见神经退行性疾病,包括阿尔茨海默病和帕金森病。这些疾病与朊病毒疾病有共同的特征,在朊病毒疾病中,错误折叠的蛋白质可以自我复制,并在不同宿主之间传播疾病。阐明导致聚集物扩增的分子机制对于理解病理性沉积物如何在大脑中传播并导致疾病至关重要。在这里,我们使用单分子显微镜在单个聚集体水平上研究了 tau 的组装和复制。我们发现 tau 聚集体具有通过丝状断裂进行自我扩增的内在能力,并通过动力学建模确定了该复制过程的倍增时间。然后,我们模拟了聚集体在大脑中的扩散时间,发现这与在阿尔茨海默病中观察到的病理性 tau 沉积物的扩散时间框架以及在 tau 病的实验模型中非常吻合。通过这项工作,我们开始了解控制 tau 和其他淀粉样蛋白在人类大脑中扩散速度的物理参数。