α-突触核蛋白缺陷小鼠对毒素诱导的多系统萎缩具有抗性。
Alpha-synuclein deficient mice are resistant to toxin-induced multiple system atrophy.
作者信息
Ubhi Kiren, Rockenstein Edward, Mante Michael, Inglis Chandra, Adame Anthony, Patrick Christina, Masliah Eliezer
机构信息
Department of Neurosciences, University of California San Diego, School of Medicine, La Jolla, California 92093-0624, USA.
出版信息
Neuroreport. 2010 Apr 21;21(6):457-62. doi: 10.1097/WNR.0b013e328338ba6b.
Multiple systems atrophy (MSA) is a neurodegenerative disorder characterized by oligodendrocytic accumulations of alpha-synuclein (alphasyn). Oxidative stress is a key mechanism proposed to underlie MSA pathology. To address the role of alphasyn modifications, over and above general oxidative modifications, this study examined the effects of 3-nitropropionic acid (3NP) administration, a technique used to model MSA, in knock-out mice lacking alphasyn (alphasynKO). Although susceptible to 3NP-induced oxidative stress, alphasynKO mice display reduced neuronal loss and dendritic pathology. The alphasynKO mice are resistant to 3NP-induced motor deficits and display attenuated loss of tyrosine hydroxylase and dopamine transporter striatal immunoreactivity. The results suggest that deficits in MSA are not due to general oxidative protein modification but in addition may be related to specific alphasyn modifications.
多系统萎缩（MSA）是一种神经退行性疾病，其特征是α-突触核蛋白（α-syn）在少突胶质细胞中积聚。氧化应激是被认为是MSA病理学基础的关键机制。为了研究α-syn修饰的作用，除了一般的氧化修饰之外，本研究检测了给予3-硝基丙酸（3NP）（一种用于模拟MSA的技术）对缺乏α-syn的基因敲除小鼠（α-synKO）的影响。尽管α-synKO小鼠易受3NP诱导的氧化应激影响，但它们的神经元损失和树突病理表现有所减轻。α-synKO小鼠对3NP诱导的运动缺陷具有抗性，并且酪氨酸羟化酶和多巴胺转运体纹状体免疫反应性的损失减弱。结果表明，MSA的缺陷并非由于一般的氧化蛋白质修饰，此外可能还与特定的α-syn修饰有关。
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