Department of Cell Biology & Histology, Academic Medical Center, Amsterdam, The Netherlands.
Department of Biomedicine, University of Basel and University Hospital Basel, Basel, Switzerland.
Biochim Biophys Acta Mol Basis Dis. 2017 Mar;1863(3):793-800. doi: 10.1016/j.bbadis.2016.12.015. Epub 2016 Dec 29.
Most neurodegenerative diseases such as Alzheimer's, Parkinson's and Huntington's disease are hallmarked by aggregate formation of disease-related proteins. In various of these diseases transfer of aggregation-prone proteins between neurons and between neurons and glial cells has been shown, thereby initiating aggregation in neighboring cells and so propagating the disease phenotype. Whereas this prion-like transfer is well studied in Alzheimer's and Parkinson's disease, only a few studies have addressed this potential mechanism in Huntington's disease. Here, we present an overview of in vitro and in vivo methodologies to study release, intercellular transfer and uptake of aggregation-prone protein fragments in Huntington's disease models.
大多数神经退行性疾病，如阿尔茨海默病、帕金森病和亨廷顿病，其特征是与疾病相关的蛋白质聚集形成。在这些疾病的许多种中，已经发现聚集倾向蛋白在神经元之间以及神经元和神经胶质细胞之间转移，从而在邻近细胞中引发聚集，从而传播疾病表型。尽管这种类朊病毒转移在阿尔茨海默病和帕金森病中得到了很好的研究，但只有少数研究探讨了亨廷顿病中这种潜在的机制。在这里，我们概述了用于研究亨廷顿病模型中聚集倾向蛋白片段的释放、细胞间转移和摄取的体外和体内方法。
