Wang Xinzhu, Noroozian Zeinab, Lynch Madelaine, Armstrong Nicholas, Schneider Raphael, Liu Mingzhe, Ghodrati Farinaz, Zhang Ashley B, Yang Yoo Jeong, Hall Amanda C, Solarski Michael, Killackey Samuel A, Watts Joel C
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.
Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, ON, Canada.
Discoveries (Craiova). 2017 Sep 30;5(3):e78. doi: 10.15190/d.2017.8.
The presence of protein aggregates in the brain is a hallmark of neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD). Considerable evidence has revealed that the pathological protein aggregates in many neurodegenerative diseases are able to self-propagate, which may enable pathology to spread from cell-to-cell within the brain. This property is reminiscent of what occurs in prion diseases such as Creutzfeldt-Jakob disease. A widely recognized feature of prion disorders is the existence of distinct strains of prions, which are thought to represent unique protein aggregate structures. A number of recent studies have pointed to the existence of strains of protein aggregates in other, more common neurodegenerative illnesses such as AD, PD, and related disorders. In this review, we outline the pathobiology of prion strains and discuss how the concept of protein aggregate strains may help to explain the heterogeneity inherent to many human neurodegenerative disorders.
大脑中蛋白质聚集体的存在是神经退行性疾病如阿尔茨海默病（AD）和帕金森病（PD）的一个标志。大量证据表明，许多神经退行性疾病中的病理性蛋白质聚集体能够自我传播，这可能使病理学在大脑内从一个细胞扩散到另一个细胞。这种特性让人联想到克雅氏病等朊病毒疾病中发生的情况。朊病毒疾病一个广泛认可的特征是存在不同的朊病毒株，这些朊病毒株被认为代表独特的蛋白质聚集体结构。最近的一些研究指出，在其他更常见的神经退行性疾病如AD、PD及相关疾病中也存在蛋白质聚集体株。在这篇综述中，我们概述了朊病毒株的病理生物学，并讨论蛋白质聚集体株的概念如何有助于解释许多人类神经退行性疾病所固有的异质性。
