8 Conicella, A. E., Zerze, G. H., Mittal, J. & Fawzi, N. L. ALS Mutations Disrupt Phase Separation Mediated by alpha-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain.Structure24, 1537-1549...
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...
Diana Arseni, et al.Structure of pathological TDP-43 filaments from ALS with FTLD. Article. 2021. Ariel Ionescu1, Topaz Altman1 and Eran Perlson. Looking for answers far away from the soma—the (un)known axonal functions of TDP-43, and their contribution to early NMJ disruption in ALS. M...
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 ...
By performing systematic energy calculations, we reveal a prevailing trend of destabilizing effects induced by these mutations in the amyloid structure, challenging the traditionally assumed correlation between pathogenicity and amyloidogenic propensity. Understanding the molecular basis of this discrepancy ...
An abundance of glycine and neutral polar residues facilitates numerous turns and restricts 尾-strand length, which results in an absence of 尾-sheet stacking that is associated with cross-尾 amyloid structure. An uneven distribution of residues gives rise to structurally and chemically distinct ...
Cryo-electron microscopy structure of the core peptide (279–360) fibrils Proteinase K-exposed TDP-43 filaments have higher seeding activity TDP-43 filament cleavage is essential for seeding in cells Amyloid core exposure boosts seeding of brain-derived TDP-43 Discussion Methods Data availability Code...
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...
An identical amyloid-like filament structure comprising a single protofilament was found in both brain regions and individuals. The ordered filament core spans residues 282–360 in the TDP-43 low-complexity domain and adopts a previously undescribed double-spiral-shaped fold, which shows no ...
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 structure formed by the TDP43 low complexity domain”的研究论文,阐述了TDP43的LCD在发生...