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货号： bs-0822R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-0822R-PE-Cy7
- 英文名称
- Anti-TARDBP/PE-Cy7
- 中文名称
- PE-Cy7标记的Tar DNA 结合蛋白43/TDP-43抗体
- 别 名
- TAR DNA-binding protein 43; TAR DNA binding protein 43; ALS10; OTTHUMP00000002171; TAR DNA binding protein 43; TAR DNA binding protein; TDP 43; TDP-43; TDP43; TARDBP; tar DNA binding protein; ALS10; TADBP_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 神经生物学 信号转导 细菌及病毒 表观遗传学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Cow, Rabbit,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 45kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human TDP-43
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
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]
Function:
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.
Subcellular Location:
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.
Tissue Specificity:
Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.
Post-translational modifications:
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.
DISEASE:
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.
Similarity:
Contains 2 RRM (RNA recognition motif) domains.
Database links:Entrez Gene: 23435Human
Entrez Gene: 230908Mouse
Entrez Gene: 298648Rat
Omim: 605078Human
SwissProt: Q13148Human
SwissProt: Q921F2Mouse
Unigene: 300624Human
Unigene: 635053Human
Unigene: 22453Mouse
Unigene: 2633Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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。