4. Arai, T. et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.Biochem. Biophys. Res. Commun.351, 602–611 (2006). 5. Arseni, D. et al. S...
这种异常包括TDP-43从细胞核转移到细胞质,并形成包涵体团块,这些团块会干扰TDP-43的正常功能,产生毒性并阻碍细胞过程,最终导致细胞死亡。 图2.轴突/NMJ中TDP-43的拟病理机制。 关于轴突TDP-43的生物学和病理作用的最新证据表明,TDP-43积聚在运动神经元的远端轴突和突触前隔室中并形成pTDP-43凝聚物。这些结构隔离...
为此,研究者分离来自野生型和TDP-43Q331K敲入小鼠的小胶质细胞并在细胞中加入ODNs,实验表明TDP-43 Q331K 不影响ODNs诱导的TDP-43斑点形成,但是,当ODNs从细胞培养基中移除后,TDP-43胞质斑点的解离受到了阻碍,这一结果似乎并不是...
The transactive response DNA binding protein 43 (TDP-43) is an RNA/DNA-binding protein that is involved in a number of cellular functions, including RNA processing and alternative splicing, RNA transport and translation, and stress granule assembly. It has attracted significant attention for being ...
The abnormal aggregation of TAR DNA-binding protein 43kDa (TDP-43) in neurons and glia is the defining pathological hallmark of theneurodegenerative disease amyotrophic lateral sclerosis (ALS) and multiple forms of frontotemporal lobar degeneration (FTLD). It is also common in other diseases, ...
Structure of TDP-43.(A)Domain Structure: The top panel (A) shows a schematic representation of TDP-43’s linear domain structure. From left to right: NTD: N-Terminal Domain; NLS: Nuclear Localization Signal; RRM1 and RRM2: RNA Recognition Motifs 1 and 2; NES: Nuclear Export Signal; Garo...
Here we used cryo-electron microscopy to determine the structures of aggregated TDP-43 in the frontal and motor cortices of an individual who had ALS with FTLD and from the frontal cortex of a second individual with the same diagnosis. An identical amyloid-like filament structure comprising a ...
Protein structure of TDP-43 The structure of TDP-43 is composed of an N-terminal domain, a nuclear localization sequence (NLS), two RNA binding domains (RBD1 and RBD2), a nuclear export signal (NES), and a C-terminal glycine rich domain (GRD) (Fig1) [34]. The protein also has an...
基于这些信息,研究人员进一步探索了TDP43 LCD是否能通过某种特定的分子机制发生相分离。从氧化还原过程中TDP43自组装过程的变化出发,于近日在PNAS杂志上发表了题为“Redox-mediated regulation of an evolutionarily conserved cross-β structur...
The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies Article Open access 13 October 2020 Structure of pathological TDP-43 filaments from ALS with FTLD Article 08 December 2021 TDP-43 forms amyloid filaments with a distinct fold...