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帕金森病中的α-突触核蛋白:致病功能及其在动物模型中的转化研究

α-Synuclein in Parkinson's disease: pathogenic function and translation into animal models.

作者信息

Eschbach Judith, Danzer Karin M

机构信息

Department of Neurology, University of Ulm, Ulm, Germany.

出版信息

Neurodegener Dis. 2014;14(1):1-17. doi: 10.1159/000354615. Epub 2013 Sep 24.

Abstract

Parkinson's disease is a common neurodegenerative disease characterised by the loss of dopaminergic neurons in the substantia nigra pars compacta and the formation of α-synuclein aggregates found in Lewy bodies throughout the brain. Several α-synuclein transgenic mouse models have been generated, as well as viral-mediated overexpression of wild-type and mutated α-synuclein to mimic the disease and to delineate the pathogenic pathway of α-synuclein-mediated toxicity and neurodegeneration. In this review, we will recapitulate what we have learned about the function of α-synuclein and α-synuclein-mediated toxicity through studies of transgenic animal models, inducible animal models and viral-based models.

摘要

帕金森病是一种常见的神经退行性疾病,其特征是黑质致密部多巴胺能神经元丧失,以及在全脑路易小体中发现α-突触核蛋白聚集体的形成。已经构建了几种α-突触核蛋白转基因小鼠模型,以及通过病毒介导野生型和突变型α-突触核蛋白的过表达来模拟该疾病,并阐明α-突触核蛋白介导的毒性和神经退行性变的致病途径。在本综述中,我们将通过对转基因动物模型、诱导性动物模型和病毒模型的研究,概括我们对α-突触核蛋白的功能以及α-突触核蛋白介导的毒性所了解的情况。

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