TDP-43 Recombinant Mouse mAb (一抗) - WB,IHC-P,IHC-F,IF,Flow-Cyt,ICC/IF | Bioss

2026-05-01~2026-06-30,AB2605
TDP-43 Recombinant Mouse mAb (一抗) - WB,IHC-P,IHC-F,IF,Flow-Cyt,ICC/IF | Bioss
货号:bsm-60381M
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概述

产品编号
bsm-60381M
产品类型
重组小鼠单抗、mIHC精品抗体
英文名称
TDP-43 Recombinant Mouse mAb
中文名称
Tar DNA 结合蛋白43/TDP-43重组小鼠单抗
英文别名
ALS10; TDP-43; 1190002A23Rik; Tdp43; TADBP_HUMAN; TARDBP; TADBP_MOUSE; TAR DNA binding protein
抗体来源
Mouse
亚型
IgG1, Kappa
性状
Liquid
纯化方法
affinity purified by Protein G
克隆类型
Recombinant
克隆号
G6A6
检测分子量
45 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍
变异蛋白质TDP-43 在额颞叶退行性病变(FTLD-U)和萎缩性侧索硬化症(ALS)中表达较高。TDP-43在大脑中堆积能导致神经细胞衰竭,从而引发疾病肌萎缩性侧索硬化(ALS,也被称为Lou Gehrig氏病)
TDP-43这种痴呆是由大脑额叶的退化引起的,退化能延伸到颞叶。这是仅次于阿尔默海兹症的让65岁以下患者痴呆的第二种最常见的原因,通常影响40几岁和50几岁的人。TDP-43过去在神经退化疾病患者病理的错误折叠蛋白中缺失。识别出这个蛋白应该有助于痴呆以及运动神经元疾病的研究。
背景资料
HIV-1, the causative agent of acquired immunodeficiency syndrome (AIDS), contains an RNA genome that produces a chromosomally integrated DNA during the replicative cycle. Activation of HIV-1 gene expression by the transactivator Tat is dependent on an RNA regulatory element (TAR) located downstream of the transcription initiation site. The protein encoded by this gene is a transcriptional repressor that binds to chromosomally integrated TAR DNA and represses HIV-1 transcription. In addition, this protein regulates alternate splicing of the CFTR gene. A similar pseudogene is present on chromosome 20. [provided by RefSeq, Jul 2008]
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产品应用

应用已检合格种属预测种属推荐稀释比例
WBHumanMouse, Rat1:1000-5000
IHC-PHuman, Mouse, Rat1:100-500
IHC-FHuman, Mouse, Rat1:100-500
IFHuman, Mouse, Rat1:100-500
Flow-CytHumanMouse, Rat1:50-100
ICC/IFHumanMouse, Rat1:100-500

交叉反应

交叉反应: Human, Mouse, Rat

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靶标

基因名
TARDBP
蛋白名
TAR DNA-binding protein 43
亚细胞定位
Nucleus. In patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis, it is absent from the nucleus of affected neurons but it is the primary component of cytoplasmic ubiquitin-positive inclusion bodies.
组织特异性
Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.
翻译后修饰
Hyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Ubiquitinated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Cleaved to generate C-terminal fragments in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
疾病
Defects in TARDBP are the cause of amyotrophic lateral sclerosis type 10 (ALS10) [MIM:612069]. ALS is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of ALS is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
相似性
Contains 2 RRM (RNA recognition motif) domains.
功能
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.

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