不过,通过ALS病理研究可发现,TDP-43蛋白异常是该病患者病变区神经元普遍发生的现象。神经元是大脑中上传下达的基本单元,也是神经退行性疾病发生与进展的关键单元。据报道,相当多的神经退行性疾病患者——97%的ALS、45%的额颞叶变性痴呆...
TDP-43蛋白作为ALS病理研究的焦点,其功能缺陷与ALS的病因及发病机制的关系仍未完全阐明。 当地时间2021年1月4日,来自加州大学圣地亚哥分校付向东和武汉大学周宇共同通讯在《Nature Structural & Molecular Biology》在线发表题为“TDP-43 aggregation induced by oxida...
Despite intensive research, the mechanisms behind protein aggregate formation in ALS remains unclear. We have investigated the role of metabolic stress in protein aggregate formation analyzing how it is relevant to the co-aggregation observed between RGNEF and TDP-43 in motor neurons of ALS patients...
ALS are characterized by the aggregation of the essential DNA- and RNA-binding protein TDP-43, suggesting a central role in ALS etiology. Here we report that TDP-43 aggregation in neuronal cells of mouse and human origin causes sensitivity to oxidative stress. Aggregated TDP-43 sequesters ...
: TAR DNA binding protein 43 (TDP-43) is aggregated as neuronal inclusions in frontotemporal lobar degeneration with ubiquitin immunoreactive inclusions (FTLD-U) and in amyotrophic lateral sclerosis (ALS), and it has been considered that TDP-43 may be a specific marker for FTLD-U and ALS. ...
大家都知道,DNA中包含4种碱基,腺嘌呤(A)、胸腺嘧啶(T)、鸟嘌呤(G)和胞嘧啶(C)组成,有时候核苷酸会重复扩增,扩增过多时就会导致一些神经系统疾病,例如肌萎缩侧索硬化症(ALS)的主要遗传因素就是C9ORF72基因中GGGGCC六核苷酸重复序列的扩增[1],亨廷顿氏病和多种形式的脊髓小脑性共济失调则与CAG重复扩增有关[2]...
相关研究结果近期发表在Science Translational Medicine期刊上，论文标题为“C9orf72 poly(GR) aggregation induces TDP-43 proteinopathy”。特别地，毒性的poly(GR)蛋白以RNA不依赖的方式介导全长TDP-43的封存，从而诱导细胞质TDP-43包涵体形成。此外，在GFP-(GR)200小鼠中，poly(GR)引起核质转运因子（nucleocytoplasmic...
A robust TDP-43 knock-in mouse model of ALS[ J]. Acta Neuropathologica Communications, 2020, 8(1): 3. 48. Ormeno F, Hormazabal J, Moreno J, et al. Chaperone mediated autophagy degrades TDP-43 protein and is affected by TDP-43 aggregation[ J]. Front Mol Neurosci, 2020, 13: 19....
TAR RNA/DNA-binding protein 43kDa (TDP43) is an ALS-causative p... A Kitamura,Y Nakayama,S Ai,... - 《Scientific Reports》 被引量: 32发表: 0年 A high-content screen identifies novel compounds that inhibit stress-induced TDP-43 cellular aggregation and associated cytotoxicity. TDP-43 is...
The Relationship Between TDP-43 Protein Aggregation and ALS Transactive response (TAR) DNA-binding protein 43 (TDP-43), encoded by theTARDBPgene, is a nuclear protein involved in the processing, transport, and metabolism of RNA, as well as the regulation of protein entry into the nucleus, cir...