3 Babinchak, W. M.et al.The role of liquid-liquid phase separation in aggregation of the TDP-43 low-complexity domain.J Biol Chem294, 6306-6317 (2019). https://doi.org:10.1074/jbc.RA118.007222 4 Carey, J....
TDP-43的氨基端部分包含一个DIX(dishevelled and axin)结构域,一个核定位信号,一个串联RNA识别基序(RNA-recognition motifs,RRMs)。而TDP-43的羧基端则主要由一个固有无序低复杂度结构域(low-complexity domain,LCD)组成,里面包含一些甘氨酸、疏水性残基以及...
N末端与TDP-43自身相互作用有关;NLS和NES与TDP-43的核质穿梭相关;富含甘氨酸的C-端序列也被称作低复杂序列结构域 (LCD)或类朊蛋白结构域 (prion-like domain),通过LCD结构域,TDP-43可以发生分子间相互作用并组装成相分离的液态凝聚体。 图1:TDP-43蛋白结构(图源参考文献1) 正常的TDP-43是怎样的? 在正常生理...
V (2016) The TDP-43 N-terminal domain structure at high resolution. FEBS Lett. 283, 1242-1260Mompean, M. et al. The TDP-43 N-terminal domain structure at high resolution. FEBS J 283, 1242-1260 (2016).Mompean, M. et al. The TDP-43 N-terminal domain structure at high resolution. ...
The majority of TDP43 protein found in cytoplasmic inclusions is truncated, and it has been shown that the C-terminal domain is intrinsically prone to aggregation. Mutations in the C-terminal region of the TDP43 gene have been associated with both ALS and FTLD, and are thought to facilitate ...
Fig. 1 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 Sig...
TDP-43 exhibits a long self-adhesive domain of low complexity (LCD) in its C-terminus that allows the formation of reversible cellular compartments with liquid-like properties3,4,5 (Fig. 1a). Additional contributions also come from its structured self-associating N-terminal domain6 and the ...
Fig. 1 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 Sig...
Fig. 1 Structure of TDP-43 protein. Numbers represent amino acid lengths of TDP-43. NLS: nuclear localization signal, RRM: RNA recognition motifs, NES: nuclear export signal, Q/N: glutamine/asparagine-rich domain, M1–M5: five putative mitochondrial localization signals ...
The RNA binding protein TDP-43 forms cytoplasmic inclusions via its C-terminal prion-like domain in several neurodegenerative diseases. Aberrant TDP-43 aggregation arises upon phase de-mixing and transitions from liquid to solid states, following still u