Arduíno Daniela M, Esteves A Raquel, Swerdlow Russell H, Cardoso Sandra M
Department of Chemistry and Biochemistry, Gene Center Munich, Ludwig-Maximilians-Universität München, Feodor-Lynen-Str. 25, 81377, Munich, Germany,
Methods Mol Biol. 2015;1265:415-24. doi: 10.1007/978-1-4939-2288-8_31.
Parkinson's disease (PD) is a multifactorial and clinically complex age-related movement disorder. The cause of its most common form (sporadic PD, sPD) is unknown, but one prominent causal factor is mitochondrial dysfunction. Although several genetic- and toxin-based models have been developed along the last decades to mimic the pathological cascade of PD, cellular models that reliably recapitulate the pathological features of the neurons that degenerate in PD are scarce.We describe here the generation of cytoplasmic hybrid cells (or cybrids) as a cellular model of sPD. This approach consists on the fusion of platelets harboring mtDNA from sPD patients with cells in which the endogenous mtDNA has been depleted (Rho0 cells).The sPD cybrid model has been successful in recapitulating most of the hallmarks of sPD, constituting now a validated model for addressing the link between mitochondrial dysfunction and sPD pathology.
帕金森病（PD）是一种多因素导致的、临床上复杂的与年龄相关的运动障碍。其最常见形式（散发性帕金森病，sPD）的病因尚不清楚，但一个突出的致病因素是线粒体功能障碍。尽管在过去几十年中已经开发了几种基于基因和毒素的模型来模拟帕金森病的病理级联反应，但能够可靠地重现帕金森病中退化神经元病理特征的细胞模型却很少。我们在此描述了细胞质杂种细胞（或细胞融合体）作为sPD细胞模型的产生。这种方法是将携带sPD患者线粒体DNA的血小板与内源性线粒体DNA已被耗尽的细胞（ρ0细胞）融合。sPD细胞融合体模型已成功重现了sPD的大部分特征，现在已成为一个经过验证的模型，用于研究线粒体功能障碍与sPD病理之间的联系。
