中国科技核心期刊
CN:31-1600/Q
ISSN:1004-0374
“健康与疾病的免疫”国际学术研讨会通知      关于有网站冒充本刊网站的声明
《生命科学》 2014, 26(7): 739-744
TDP-43在神经退行性疾病中的功能和作用
刘 丽,申景岭*
(哈尔滨医科大学基础医学院组织学与胚胎学教研室,哈尔滨 150081)
摘 要:核蛋白TAR DNA/RNA 结合蛋43 (TDP-43) 目前被认为是肌萎缩侧索硬化症(amyotrophic lateral sclerosis, ALS)、额颞叶变性(frontotemporal lobar degeneration, FTLD) 等神经退行性疾病的病理学标记蛋白。在中枢神经系统中,TDP-43 作为必要的转录调控因子,参与mRNA 前体的剪接,维持RNA 稳态和运输。在突变和过表达TDP-43 的转基因啮齿类动物模型中,受损伤的神经元呈现出胞核和胞质中TDP-43 泛素化、磷酸化聚集,以及细胞周期进程的改变。在此,着重阐述基于TDP-43 突变或过表达建立神经退行性疾病动物模型的研究进展,探讨其发病机制、病理学改变及治疗方法。
 
The function of TDP-43 in neurodegenerative disease
LIU Li, SHEN Jing-Ling*
(Department of Histology and Embryology, Basic Medical Science College,
    Harbin Medical University, Harbin 150081, China)
Abstract: Nuclear protein TAR DNA /RNA binding protein 43 (TDP-43) is recognized as a pathological marker protein of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). It is identified as a regulator of essential transcriptional events in the CNS, which is involved in pre-mRNA splicing, RNA stability and transport. In affected neurons of transgenic rodent models with TDP-43 mutation or overexpression, nuclear and cytoplasmic TDP-43 aggregates with ubiquitination or phosphorylation, and cell cycles also alterate. Here research progress on transgenic rodent models made by TDP-43 mutation or overexpression is reviewed to explore molecular mechanisms, pathological changes and new therapies for neurodegenerative disease.
 
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