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Tar DNA 结合蛋白43抗体费用 英文名称  Anti-TDP-43/TARDBP

中文名称  Tar DNA 结合蛋白43抗体费用 

别    名 TAR DNA-binding protein 43; ALS10; OTTHUMP00000002171; TAR DNA binding protein 43; TAR DNA binding protein; TDP 43; TDP43; TARDBP; tar DNA binding protein.

产品属性：

浓    度  1mg/1ml

规 格  0.1ml/100μg 0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Chicken, Cow, Rabbit

产品类型   一抗  

研究领域    神经生物学 信号转导 细菌及 表观遗传学

蛋白分子量  predicted molecular weight: 45kDa 

性    状   Lyophilized or Liquid

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 PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

Tar DNA 结合蛋白43抗体费用 产品应用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品介绍 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 -1 transcription by binding to the -1 long terminal repeat. [Subcellular Location] Nucleus. Note=Eliminated from nuclei of ubiquitinated inclusion-bearing neurons in FTLDU. [Tissue Specificity] Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.

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]

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.

变异蛋白质TDP-43 在额颞叶退行性（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过去在疾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以及运动神经元疾病的研究。

实验流程：

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