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散发性帕金森病中路易小体内的TorsinA积聚。

TorsinA accumulation in Lewy bodies in sporadic Parkinson's disease.

作者信息

Shashidharan P, Good P F, Hsu A, Perl D P, Brin M F, Olanow C W

机构信息

Department of Neurology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA.

出版信息

Brain Res. 2000 Sep 22;877(2):379-81. doi: 10.1016/s0006-8993(00)02702-5.

DOI:10.1016/s0006-8993(00)02702-5
PMID:10986355
Abstract

Parkinson's disease (PD) is a neurodegnerative disorder that is pathologically characterized by the presence of Lewy bodies in the brain. We show that Lewy bodies in PD are strongly immunoreactive for torsinA, the protein product of the DYT1 gene, which is associated with primary generalized dystonia. In the substantia nigra, torsinA immunoreactivity is localized to the periphery of Lewy bodies, whereas, in cortical Lewy bodies it is uniformly distributed. The significance of this finding is unknown, but may implicate torsinA in neuronal dysfunction that occurs in PD as well as in primary dystonia.

摘要

帕金森病(PD)是一种神经退行性疾病,其病理特征是大脑中存在路易小体。我们发现,帕金森病中的路易小体对扭转蛋白A具有强烈的免疫反应性,扭转蛋白A是DYT1基因的蛋白质产物,与原发性全身性肌张力障碍有关。在黑质中,扭转蛋白A免疫反应性定位于路易小体的周边,而在皮质路易小体中,它是均匀分布的。这一发现的意义尚不清楚,但可能意味着扭转蛋白A参与了帕金森病以及原发性肌张力障碍中发生的神经元功能障碍。

相似文献

1
TorsinA accumulation in Lewy bodies in sporadic Parkinson's disease.散发性帕金森病中路易小体内的TorsinA积聚。
Brain Res. 2000 Sep 22;877(2):379-81. doi: 10.1016/s0006-8993(00)02702-5.
2
Nigral and cortical Lewy bodies and dystrophic nigral neurites in Parkinson's disease and cortical Lewy body disease contain alpha-synuclein immunoreactivity.帕金森病和皮质路易体病中的黑质和皮质路易小体以及营养不良性黑质神经突含有α-突触核蛋白免疫反应性。
J Neuropathol Exp Neurol. 1998 Apr;57(4):334-7. doi: 10.1097/00005072-199804000-00005.
3
[The mechanism of Lewy body formation in Parkinson's disease].[帕金森病中路易小体形成的机制]
Nihon Rinsho. 2000 Oct;58(10):2022-7.
4
A close association of torsinA and alpha-synuclein in Lewy bodies: a fluorescence resonance energy transfer study.路易小体中torsinA与α-突触核蛋白的紧密关联:一项荧光共振能量转移研究。
Am J Pathol. 2001 Jul;159(1):339-44. doi: 10.1016/s0002-9440(10)61700-2.
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Synphilin-1 and parkin show overlapping expression patterns in human brain and form aggresomes in response to proteasomal inhibition.α-突触核蛋白-1和帕金蛋白在人类大脑中呈现出重叠的表达模式,并在蛋白酶体抑制时形成聚集体。
Neurobiol Dis. 2005 Nov;20(2):401-11. doi: 10.1016/j.nbd.2005.03.021.
6
Proteomic approach to studying Parkinson's disease.蛋白质组学方法在帕金森病研究中的应用
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7
Lewy-body formation is an aggresome-related process: a hypothesis.路易小体形成是一个与聚集体相关的过程:一种假说。
Lancet Neurol. 2004 Aug;3(8):496-503. doi: 10.1016/S1474-4422(04)00827-0.
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Controversy: is Parkinson's disease a single disease entity? Yes.争议:帕金森病是单一的疾病实体吗?答案是肯定的。
Parkinsonism Relat Disord. 2005 Jun;11 Suppl 1:S31-7. doi: 10.1016/j.parkreldis.2005.02.011. Epub 2005 Apr 20.
9
TorsinA and heat shock proteins act as molecular chaperones: suppression of alpha-synuclein aggregation.扭转蛋白A和热休克蛋白作为分子伴侣:抑制α-突触核蛋白聚集。
J Neurochem. 2002 Nov;83(4):846-54. doi: 10.1046/j.1471-4159.2002.01190.x.
10
Synphilin-1 is present in Lewy bodies in Parkinson's disease.α-突触核蛋白-1存在于帕金森病的路易小体中。
Ann Neurol. 2000 Apr;47(4):521-3.

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Tremor Other Hyperkinet Mov (N Y). 2014 Sep 23;4:260. doi: 10.7916/D8JS9NR2. eCollection 2014.
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