货号：MABN150

品牌：Merck Millipore

规格：

目录价：￥4755.00

市场价格：￥4041.75

会员价格：￥3804.00

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-TAR DNA binding protein-43 Antibody, clone TAR5 2H4 | MABN150 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: ~47 kDa observed. Uniprot describes 3 isoforms at 28 kDa, 44 kDa, and 45 kDa. View All» Immunogen: Ovalbumin-conjugated linear peptide corresponding to human TAR DNA binding protein-43. View All» Clone: TAR5 2H4 View All» Isotype: IgG2aκ View All» Background Information: 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. View All» Species Reactivity: Human View All» Species Reactivity Note: Demonstrated to react with Human. View All» Application Notes: 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. View All» Control: HeLa nuclear extract View All» Quality Assurance: 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. View All» Purification Method: Protein G purified View All» Presentation: Purified rat monoclonal IgG2aκ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q13148 View All» Entrez Gene Number: NP_031401 View All» Gene Symbol:

• TARDBP

• TDP43
  View All» Alternate Names:
  • TAR DNA-binding protein 43

  • TDP-43
    View All» Usage Statement: 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. View All» Key Applications: Western Blotting View All» Entrez Gene Summary: 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]. View All» UniProt Summary: 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. View All» Product Name: Anti-TAR DNA binding protein-43, clone TAR5 2H4 View All» Concentration: 1 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Rat View All»
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