摘要
亨廷顿氏病(Huntington s disease,HD)是一类神经系统退行性疾病,主要病理改变累及基底节神经元以及纹状体的固有神经元,患者主要临床特征为进行性恶化的运动、认知及精神障碍三联征。3-硝基丙酸(3-NP)是线粒体毒性药物,主要作用于氧化呼吸链并抑制机体的三羧酸循环。本文将就HD的发病机制及病理学特征以及3-NP介导的动物模型的优缺点做一综述。
Huntington＇s disease （HD） is a progressive neurodegenerative disorder associated with severe degeneration of basal ganglia neurons, especially the intrinsic neurons of the striatum, and characterized by progressive aggravating triad： uncontrolled movement, disturbance in cognition and decline in some mental abilities. 3-nitropropionic acid （3-NP）, an inhibitor of the mitochondrial citric acid cycle, results in a progressive locomotor deterioration resembling that of HD. The article below will present the pathological features and pathogenesis of Huntington＇s disease and the origin, influencing factor and both the advantage and disadvantage of 3-NP-induced animal model of HD.
出处
《中国比较医学杂志》
CAS
2009年第8期70-75,共6页
Chinese Journal of Comparative Medicine
关键词
亨廷顿氏病
3-硝基丙酸
模型
动物
Huntington＇s disease （HD）
3-nitropropionic acid （3-NP）
animal model
