Neuronal Survival Unit, Wallenberg Neuroscience Center, Lund University, Lund, Sweden.
J Clin Invest. 2011 Feb;121(2):715-25. doi: 10.1172/JCI43366. Epub 2011 Jan 18.
Post-mortem analyses of brains from patients with Parkinson disease who received fetal mesencephalic transplants show that α-synuclein-containing (α-syn-containing) Lewy bodies gradually appear in grafted neurons. Here, we explored whether intercellular transfer of α-syn from host to graft, followed by seeding of α-syn aggregation in recipient neurons, can contribute to this phenomenon. We assessed α-syn cell-to-cell transfer using microscopy, flow cytometry, and high-content screening in several coculture model systems. Coculturing cells engineered to express either GFP- or DsRed-tagged α-syn resulted in a gradual increase in double-labeled cells. Importantly, α-syn-GFP derived from 1 neuroblastoma cell line localized to red fluorescent aggregates in other cells expressing DsRed-α-syn, suggesting a seeding effect of transmitted α-syn. Extracellular α-syn was taken up by cells through endocytosis and interacted with intracellular α-syn. Next, following intracortical injection of recombinant α-syn in rats, we found neuronal uptake was attenuated by coinjection of an endocytosis inhibitor. Finally, we demonstrated in vivo transfer of α-syn between host cells and grafted dopaminergic neurons in mice overexpressing human α-syn. In summary, intercellularly transferred α-syn interacts with cytoplasmic α-syn and can propagate α-syn pathology. These results suggest that α-syn propagation is a key element in the progression of Parkinson disease pathology.
对接受胎脑移植的帕金森病患者的大脑进行死后分析显示,含有α-突触核蛋白(α-synuclein-containing,α-syn-containing)的路易体(Lewy bodies)逐渐出现在移植的神经元中。在这里,我们探讨了宿主细胞到移植物的α-syn 细胞间转移,以及随后在受者神经元中α-syn 聚集的播种是否有助于这一现象。我们使用显微镜、流式细胞术和高内涵筛选在几种共培养模型系统中评估了α-syn 的细胞间转移。用 GFP-或 DsRed 标记的α-syn 工程化细胞共培养导致双标记细胞逐渐增加。重要的是,源自 1 个神经母细胞瘤细胞系的α-syn-GFP 定位于表达 DsRed-α-syn 的其他细胞中的红色荧光聚集体,提示传递的α-syn 具有播种效应。细胞通过内吞作用摄取细胞外 α-syn,并与细胞内的α-syn 相互作用。接下来,在大鼠脑内注射重组α-syn 后,我们发现通过共注射内吞抑制剂可减弱神经元摄取。最后,我们在过表达人α-syn 的小鼠中证明了宿主细胞和移植多巴胺能神经元之间的体内α-syn 转移。总之,细胞间转移的α-syn 与细胞质中的α-syn 相互作用,并可传播α-syn 病理学。这些结果表明,α-syn 传播是帕金森病病理学进展的关键因素。