Institute for Neurodegenerative Diseases, Department of Neurology, Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, California 94158.
F. Edward Hébert School of Medicine, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814.
Cold Spring Harb Perspect Biol. 2017 Nov 1;9(11):a023549. doi: 10.1101/cshperspect.a023549.
Although the discovery of the prion protein (PrP) resulted from its co-purification with scrapie infectivity in Syrian hamsters, work with genetically defined and genetically modified mice proved crucial for understanding the fundamental processes involved not only in prion diseases caused by PrP misfolding, aggregation, and spread but also in other, much more common, neurodegenerative brain diseases. In this review, we focus on methodological and conceptual approaches used to study scrapie and related PrP misfolding diseases in mice and how these approaches have advanced our understanding of related disorders including Alzheimer's and Parkinson's disease.
虽然朊病毒蛋白(PrP)的发现源于其与叙利亚仓鼠中的传染性瘙痒症共纯化,但使用遗传定义和遗传修饰的小鼠进行的研究对于理解不仅涉及由 PrP 错误折叠、聚集和传播引起的朊病毒疾病,而且还涉及其他更为常见的神经退行性脑疾病的基本过程至关重要。在这篇综述中,我们专注于用于研究小鼠中的瘙痒症和相关 PrP 错误折叠疾病的方法学和概念方法,以及这些方法如何促进我们对包括阿尔茨海默病和帕金森病在内的相关疾病的理解。