Laboratory of Molecular Biotechnology, University of Science, VNU-HCM, 227 Nguyen Van Cu St, Dist 5, Ho Chi Minh City, Vietnam,
Front Biosci (Landmark Ed). 2020 Jan 1;25(1):159-167. doi: 10.2741/4800.
Parkinson's disease (PD) is a neurodegenerative disease caused by genetic or environmental factors. Among several animal models, the is one of the valuable models widely used in studying genes and proteins implicated in PD. UCH-L1 (Ubiquitin carboxyl-terminal hydrolase L1) which is involved in formation of Lewy bodies, shows loss of function mutations in PD causing degeneration of dopaminergic neurons in mice. Here, we summarize the results from studying the UCH-L1 and its knockdown in model of PD with respect to movement, degeneration of dopamine producing neurons, dopamine deficiency and age dependent dependency of progression of the disease. The knockdown of the UCH-L1 in can be used in studying the epidemiology of the disease as well as in drug screening for finding therapeutic targets for PD.
帕金森病(PD)是一种由遗传或环境因素引起的神经退行性疾病。在几种动物模型中, 是研究与 PD 相关的基因和蛋白质的有价值的模型之一。参与路易体形成的泛素羧基末端水解酶 L1(UCH-L1)在 PD 中存在功能丧失突变,导致小鼠多巴胺能神经元变性。在这里,我们总结了在 PD 模型中研究 UCH-L1 及其敲低对运动、多巴胺能神经元变性、多巴胺缺乏以及疾病进展对年龄依赖性的影响的结果。在 中敲低 UCH-L1 可用于研究该疾病的流行病学,以及用于筛选治疗 PD 的治疗靶点的药物。