TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein
TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein
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TDP43 是一种人类低分子量神经丝蛋白（hNFL）mRNA 结合蛋白
DOI：
10.1016/j.mcn.2007.03.007
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发表时间：
2007-06-01
影响因子：
3.5
通讯作者：
Shoesmith, Christen
中科院分区：
文献类型：
--
作者：
Strong, Michael J.;Volkening, Kathryn;Shoesmith, Christen
人TAR DNA结合蛋白（TDP 43）与肌萎缩侧索硬化症（ALS）运动神经元中的泛素化包涵体共定位。TDP 43是一种DNA结合蛋白，其核输出序列与TG、T. DNA中的元件和与单链RNA中的（UG）（6-12）基序相互作用的RNA结合蛋白。在对照运动神经元中，TDP 43几乎完全是核的，而在ALS脊髓运动神经元中，TDP 43主要定位于细胞质而不是细胞核。TDP 43被观察为点状免疫反应性和致密的绞链，有和没有泛素化。我们观察到TDP 43通过与3 'UTR的直接相互作用稳定人低分子量（hNFL）mRNA。TDP 43是一种独特的hNFL mRNA结合蛋白，其在ALS脊髓运动神经元中的躯体位置定位发生改变，并可能通过改变NF化学计量而有助于ALS中NF聚集体的形成。(c)2007爱思唯尔公司All rights reserved.
The human TAR DNA-binding protein (TDP43) colocalizes with ubiquitinated inclusions in motor neurons in amyotrophic lateral sclerosis (ALS). TDP43 is both a DNA-binding protein with a nuclear export sequence that interacts with (TG),T. elements in DNA and an RNA-binding protein that interacts with (UG)(6-12) motifs in single-stranded RNA. In control motor neurons, TDP43 was almost exclusively nuclear, whereas in ALS spinal motor neurons, TDP43 was predominantly localized to the cytosol and not the nucleus. TDP43 was observed as punctuate immunoreactivity and as dense skeins, with and without ubiquitinization. We observed that TDP43 stabilizes the human low molecular weight (hNFL) mRNA through a direct interaction with the 3'UTR. TDP43 is a unique hNFL mRNA-binding protein that is altered in its somatotopic localization in ALS spinal motor neurons and potentially contributes to the formation of NF aggregates in ALS through alterations in NF stoichiometry. (c) 2007 Elsevier Inc. All rights reserved.