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merck millipore,默克密理博,MABN150,Anti-TAR DNA binding protein-43 Antibody, clone TAR5 2H4
产品名称：Anti-TAR DNA binding protein-43 Antibody, clone TAR5 2H4
产品型号：MABN150
Detect TAR DNA binding protein-43 using this Anti-TAR DNA binding protein-43 Antibody, clone TAR5 2H4 validated for use in Western Blotting.
merck millipore,默克密理博,MABN150,Anti-TAR DNA binding protein-43 Antibody, clone TAR5 2H4
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H WB R Purified Monoclonal Antibody 描述 产品目录编号 MABN150 描述 Anti-TAR DNA binding protein-43 Antibody, clone TAR5 2H4 Alternate Names - TAR DNA binding protein-43
- TAR DNA-binding protein 43
- TDP-43
- TDP43
背景信息 Tar DNA-binding protein 43 (TDP-43) is a nuclear protein that binds both DNA and RNA at its RNA-recognition motifs and regulates RNA transcription and splicing. It is highly abundant in the lungs, genital tract, spleen, pancreas, and placenta. Tar DNA-binding protein 43 plays an important role in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. In both conditions, Tar DNA-binding protein 43 is found in the cytoplasm of the hippocampus, neocortex and spinal cord, only in a hyperphosphorylated form that is marked for ubiquitin-mediated degradation. 产品信息 格式 Purified 控制 - HeLa nuclear extract
演示 Purified rat monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Detect TAR DNA binding protein-43 using this Anti-TAR DNA binding protein-43 Antibody, clone TAR5 2H4 validated for use in Western Blotting. 主要应用 - Western Blotting
应用说明 Western Blot Analysis: A 1:1,000 dilution from a representative lot detected TAR DNA binding protein-43 in post mortem human brain tissue from a FTLD-TDP patient. Data courtesy from the laboratory of Dr. Manuela Neumann, Institute of Neuropathology, Switzerland. 生物信息 免疫原品种 Ovalbumin-conjugated linear peptide corresponding to human TAR DNA binding protein-43. 克隆 TAR5 2H4 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rat 同种型 IgG2aκ 品种反应性 Human Species Reactivity Note Demonstrated to react with Human. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_031401
Entrez基因汇总 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]. 基因符号 - TDP43
- TARDBP
纯化方法 Protein G purified UniProt编号 - Q13148
UniProt汇总 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.
SUBUNIT STRUCTURE; Binds specifically to pyrimidine-rich motifs of TAR DNA and to single stranded TG repeated sequences. Binds to RNA, specifically to UG repeated sequences with a minimun of six contiguous repeats.
SUBCELLULAR LOCATION: Nucleus. Note: 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 SPECIFICTY: Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.
PTM: 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.
INVOLVEMENT IN 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.
SEQUENCE SIMILARITIES: Contains 2 RRM (RNA recognition motif) domains.产品使用声明 质量保证 Evaluated by Western Blot in HeLa nuclear extract.
Western Blot Analysis: 0.2 µg/mL of this antibody detected TAR DNA binding protein-43 on 10 µg of HeLa nuclear extract.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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