Pael-R is accumulated in Lewy bodies of Parkinson's disease

Pael-R is accumulated in Lewy bodies of Parkinson's disease
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DOI:
10.1002/ana.20064
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发表时间:
2004-03-01
影响因子:
11.2
通讯作者:
Abe, K
Abe, K
中科院分区:
医学1区
文献类型:
--
作者:
Murakami, T;Shoji, M;Abe, K

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我们研究了帕金森病(PD)和多系统萎缩症(MSA)中PAEL-R的分布,PAEL-R是一种新发现的帕金森病底物。Pael-R、Parkin、α-突触核蛋白和泛素在路易体(Lbs)和轴突中聚集。PAEL-R定位于下丘脑核心区。Parkin和α-突触核蛋白聚集在晕层、神经元胞体和突起中。这些发现可能提示PAEL-R参与了LB的形成,以及Parkin在PAEL-R介导的帕金森病神经毒性中的保护作用。MSA中胶质细胞质内含物(GCIS)中PAEL-R和Parkin的缺失提示LBS和GCIS的形成有不同的途径。
We examined the distribution of Pael-R, a newly identified substrate for Parkin, in Parkinson's disease (PD) and multiple system atrophy (MSA). Pael-R, Parkin, a-synuclein, and ubiquitin accumulated in Lewy bodies (LBs) and neurites. Pael-R was localized in the core of LBs. Parkin and a-synuclein accumulated in the halo, neuronal cell bodies, and processes. These findings potentially suggest the involvement of Pael-R in LB formation, and protection role of Parkin in Pael-R-mediated neurotoxicity in PD. The absence of Pael-R and Parkin in glial cytoplasmic inclusions (GCIs) in MSA implies a distinct pathway involved in the formation of LBs and GCIs.