2 Ratti, A. & Buratti, E. Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.J Neurochem138 Suppl 1, 95-111 (2016). https://doi.org:10.1111/jnc.13625 3 Babinchak, W. M.et al.The rol...
7.Swarup, V., et al., Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments. Brain, 2011. 134(9): p. 2610-2626. 8.Liu, R...
2. B. S. Johnson et al., TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. J. Biol. Chem. 284, 20329–20339 (2009). doi: 10.1074/jbc.M109.010264;pmid: 19465477 3. T. Arai et al., TDP-43 is a com...
liquid-like phase and prevents it from amyloid aggregation 的论文，发现分子伴侣Hsp70是维持TDP-43在细胞应激下的液-液相分离稳态、抑制TDP-43病理性聚集的关键分子，并进一步揭示了Hsp70通过结合TDP-43上保守的聚集核心区域行使该调控功能的分子机制。该
Protein aggregation plays key roles in age-related degenerative diseases, but how different proteins coalesce to form inclusions that vary in composition, morphology, molecular dynamics and confer physiological consequences is poorly understood. Here we employ a general reporter based on mutant Hsp104 to...
Despite the strong evidence linking the transactive response DNA-binding protein 43 (TDP-43) aggregation to the pathogenesis of frontotemporal lobar degeneration with TDP-43, amyotrophic lateral sclerosis and several neurodegenerative diseases, our knowl
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 ...
Kumita, et al., Flow cytometric measurement of the cellular propagation of TDP-43 aggregation, Prion 11 (2017) 195-204, http://dx.doi.org/10.1080/ 19336896.2017.1314426.Zeineddine R, Whiten DR, Farrawell NE, McAlary L, Hanspal MA, Kumita JR, Wilson MR, Yerbury JJ (2017) Flow ...
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. Metabolic stress was able to induce formation of micronuclei, small nuclear fragments, in cultured...
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...