脑内接种病理性 α-突触核蛋白会在小鼠中引发快速进行性神经退行性 α-突触核蛋白病。

Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice.

机构信息

Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

出版信息

J Exp Med. 2012 May 7;209(5):975-86. doi: 10.1084/jem.20112457. Epub 2012 Apr 16.

Abstract

The accumulation of misfolded proteins is a fundamental pathogenic process in neurodegenerative diseases. However, the factors that trigger aggregation of α-Synuclein (α-Syn), the principal component of the intraneuronal inclusions known as Lewy bodies (LBs), and Lewy neurites (LNs), which characterize Parkinson's disease (PD) and dementia with LBs (DLB), are poorly understood. We show here that in young asymptomatic α-Syn transgenic (Tg) mice, intracerebral injections of brain homogenates derived from older Tg mice exhibiting α-Syn pathology accelerate both the formation of intracellular LB/LN-like inclusions and the onset of neurological symptoms in recipient animals. Pathological α-Syn propagated along major central nervous system (CNS) pathways to regions far beyond injection sites and reduced survival with a highly reproducible interval from injection to death in inoculated animals. Importantly, inoculation with α-Syn amyloid fibrils assembled from recombinant human α-Syn induced identical consequences. Furthermore, we show for the first time that synthetic α-Syn fibrils are wholly sufficient to initiate PD-like LBs/LNs and to transmit disease in vivo. Thus, our data point to a prion-like cascade in synucleinopathies whereby cell-cell transmission and propagation of misfolded α-Syn underlie the CNS spread of LBs/LNs. These findings open up new avenues for understanding the progression of PD and for developing novel therapeutics.

摘要

错误折叠蛋白的积累是神经退行性疾病的一个基本发病过程。然而,导致 α-突触核蛋白(α-Syn)聚集的因素,α-Syn 是称为路易体(LB)的神经元内包涵体的主要成分,以及路易神经突(LN),其特征是帕金森病(PD)和路易体痴呆(DLB),目前了解甚少。我们在这里表明,在年轻无症状的 α-Syn 转基因(Tg)小鼠中,来自表现出 α-Syn 病理学的老年 Tg 小鼠的脑匀浆的脑内注射加速了细胞内 LB/LN 样包涵体的形成和接受动物的神经症状的发作。病理性 α-Syn 通过主要中枢神经系统(CNS)途径传播到远远超出注射部位的区域,并降低了接种动物的存活率,从接种到死亡的间隔具有高度可重复性。重要的是,从重组人 α-Syn 组装的 α-Syn 淀粉样纤维的接种引起了相同的后果。此外,我们首次表明,合成的 α-Syn 纤维足以引发类似 PD 的 LB/LN 并在体内传播疾病。因此,我们的数据表明,在突触核蛋白病中存在类朊病毒级联反应,其中错误折叠的 α-Syn 的细胞-细胞传递和传播是 LB/LN 在中枢神经系统传播的基础。这些发现为理解 PD 的进展和开发新的治疗方法开辟了新的途径。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/86e6/3348112/e0ae766a5a40/JEM_20112457_Fig1.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索