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朊病毒样假说对帕金森病中聚集的α-突触核蛋白传播的解释有何可取之处?

What's to like about the prion-like hypothesis for the spreading of aggregated α-synuclein in Parkinson disease?

机构信息

Neuronal Survival Unit, Wallenberg Neuroscience Center, Lund University, Lund, Sweden.

出版信息

Prion. 2013 Jan-Feb;7(1):92-7. doi: 10.4161/pri.23806. Epub 2013 Jan 1.

Abstract

α-Synuclein is a key protein in Parkinson disease. Not only is it the major protein component of Lewy bodies, but it is implicated in several cellular processes that are disrupted in Parkinson disease. Misfolded α-synuclein has also been shown to spread from cell-to-cell and, in a prion-like fashion, trigger aggregation of α-synuclein in the recipient cell. In this mini-review we explore the evidence that misfolded α-synuclein underlies the spread of pathology in Parkinson disease and discuss why it should be considered a prion-like protein.

摘要

α-突触核蛋白是帕金森病的关键蛋白。它不仅是路易小体的主要蛋白成分,还涉及几种在帕金森病中受到干扰的细胞过程。已证明错误折叠的 α-突触核蛋白可以在细胞间传播,并以类朊病毒样的方式触发受体细胞中 α-突触核蛋白的聚集。在这篇迷你综述中,我们探讨了错误折叠的 α-突触核蛋白是如何导致帕金森病病理扩散的证据,并讨论了为什么它应该被视为类朊病毒蛋白。

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