12.Mollasalehi, N., et al., An allosteric modulator of RNA binding targeting the N-terminal domain of TDP-43 yields neuroprotective properties. ACS chemical biology, 2020. 15(11): p. 2854-2859. 13.Ni, J., et...
TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion. EMBO J. 35, 121-142.Xia Q, Wang H, Hao Z, Fu C, Hu Q, Gao F, Ren H, Chen D, Han J, Ying Z, Wang G (2015) TDP-43 loss of function increases TFEB activity and blocks autophagosome-...
We use essential cookies to make sure the site can function. We also use optional cookies for advertising, personalisation of content, usage analysis, and social media. By accepting optional cookies, you consent to the processing of your personal data - including transfers to third parties. Some...
Partial loss of TDP-43 function causes motor dysfunction without evidence of neurodegeneration. Loss of TDP-43 is associated with muscle weakness and decreased endurance without any indications of ataxia or altered reflexes. Conclusions: Both loss of TDP-43 and dominant missense mutations in TDP-43 ...
Structure and function of TDP-43 As part of the heterogeneous nuclear ribonucleoprotein (hnRNP) family, TDP-43 is primarily located in the nucleus, although it can also exist within the cytoplasm and mitochondria [10,11,12]. The structural composition of TDP-43 includes an N-terminal region wi...
Consistent with a loss of TDP-43 function, the acetylation-mimic K145Q mutant displayed a partial reduction in the spliced/ unspliced ratio relative to the baseline, and a similar but more severe loss of function was observed with the RNA-binding- deficient 4FL mutant, which completely ...
Further analyses reveal that a TDP-43 knockdown-induced reduction in GluN2A contributes to synaptic loss. Our results show that loss of TDP-43 function underlies hippocampal and cortical synaptic degeneration in TDP-43 proteinopathies. 展开
The function of TDP-43, much like other hnRNPs, is to regulate gene expression and other aspects of RNA processing including RNA splicing, mRNA turnover, RNA trafficking, and microRNA (miRNA) biogenesis [15,16,17,18,19,20,21,22]. TDP-43 targets over 4,000 different mRNA transcripts [...
关键词:TDP-43;神经退行性疾病;ALS;动物模型 中图分类号:Q426;R742.5文献标志码:A ThefunctionofTDP-43inneurodegenerativedisease LIULi,SHENJing-Ling* (DepartmentofHistologyandEmbryology,BasicMedicalScienceCollege, HarbinMedicalUniversity,Harbin150081,China) Abstract:NuclearproteinTARDNA/RNAbindingprotein43(TDP-...
TDP-43 contains four structural regions, which are an N-terminal domain, two RNA recognition motifs (RRM1 and RRM2), and a C-terminal glycine-rich region (Fig.2a). TDP-43 may function as a homodimer via the interaction of N-terminal regions20,30, but the misfolded structure of TDP-43 ...