在部分ALS和FTD患者中,TARDBP基因突变会导致TDP-43蛋白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[4-5]。更为重要的是,无论是否存在TARDBP突变,几乎所有的ALS病例中都能观察到TDP-43蛋白的病理性异常。实际上,TDP-43蛋白是ALS患者死后组织中蛋...
[8] Buratti E. Targeting TDP-43 proteinopathy with drugs and drug-like small molecules. Br J Pharmacol. 2021 Mar;178(6):1298-1315. [9] ALS News Today. (n.d.). ALS Gene Therapy SOL-257 Targeting TDP-43 Shows Promise in Mouse Model. Retrieved from https://alsnewstoday.com/news/als...
[8] Buratti E. Targeting TDP-43 proteinopathy with drugs and drug-like small molecules. Br J Pharmacol. 2021 Mar;178(6):1298-1315. [9] ALS News Today. (n.d.). ALS Gene Therapy SOL-257 Targeting TDP-43 Shows Promise in Mouse Model. Retrieved from https://alsnewstoday.com/news/als...
13.Ni, J., et al., Loss of TDP-43 function underlies hippocampal and cortical synaptic deficits in TDP-43 proteinopathies. Molecular Psychiatry, 2021: p. 1-15. 14.Brown, A.-L., et al., TDP-43 loss and ...
The findings that amyotrophic lateral sclerosis (ALS) patients almost universally display pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its gene cause familial ALS have nominated altered RNA metabolism as a disease mechanism. However, the RNAs regulated by TDP-43...
Fallini C, Bassell GJ, Rossoll W (2012) The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth . Human Molecular Genetics 21 : 3703–3718Fallini C, Bassell GJ, Rossoll W. The ALS disease protein TDP-43 is actively transported in motor ...
近日,康奈尔大学胡风华教授课题组在Journal of Biological Chemistry发表题为Regulation of TAR DNA binding protein 43 (TDP-43) homeostasis by cytosolic DNA accumulation的研究论文。该论文报道了在细胞质聚集的DNA分子与TDP-43相互...
TDP-43是一种重要的RNA结合蛋白,其基因突变是诱发ALS的原因之一【2】。 相关研究表明,在正常细胞中,TDP-43主要弥散分布于细胞核内,但可穿梭至胞浆并与其它蛋白相互作用,形成应激颗粒等各种核糖核蛋白复合物,参与RNA剪切、成熟、加工、运输、转运、翻译、降解等多个步骤的调控【3-4】;然而,在疾病状态下,TDP-43...
Disruption of ER ion homeostasis maintained by an ER anion channel CLCC1 contributes to ALS-like pathologies See more citations 相关产品 14813-1-AP FUS/TLS Monoclonal antibody 60160-1-Ig P62,SQSTM1 Polyclonal antibody 18420-1-AP TDP-43 (human specific) Monoclonal antibody ...