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UPF3B Antibody

  • 货号：
    CSB-PA883646LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA883646LA01HU diluted at 1:500 and staining in paraffin-embedded human lung cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA883646LA01HU diluted at 1:500 and staining in paraffin-embedded human colon cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of Hela cells with CSB-PA883646LA01HU at 1:166, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) UPF3B Polyclonal antibody
  • Uniprot No.：
    Q9BZI7
  • 基因名：
    UPF3B
  • 别名：
    UPF3B antibody; RENT3B antibody; UPF3X antibody; Regulator of nonsense transcripts 3B antibody; Nonsense mRNA reducing factor 3B antibody; Up-frameshift suppressor 3 homolog B antibody; hUpf3B antibody; Up-frameshift suppressor 3 homolog on chromosome X antibody; hUpf3p-X antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Regulator of nonsense transcripts 3B protein (319-423AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，UPF3B Antibody (CSB-PA883646LA01HU)，的标记方式是Non-conjugated。对于UPF3B Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA883646LB01HU UPF3B Antibody, HRP conjugated ELISA
    FITC CSB-PA883646LC01HU UPF3B Antibody, FITC conjugated
    Biotin CSB-PA883646LD01HU UPF3B Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:500-1:1000
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons by associating with the nuclear exon junction complex (EJC) and serving as link between the EJC core and NMD machinery. Recruits UPF2 at the cytoplasmic side of the nuclear envelope and the subsequent formation of an UPF1-UPF2-UPF3 surveillance complex (including UPF1 bound to release factors at the stalled ribosome) is believed to activate NMD. In cooperation with UPF2 stimulates both ATPase and RNA helicase activities of UPF1. Binds spliced mRNA upstream of exon-exon junctions. In vitro, stimulates translation; the function is independent of association with UPF2 and components of the EJC core.
  • 基因功能参考文献：
    1. The s discovered that UPF3B (i) interacts with the release factors, (ii) delays translation termination and (iii) dissociates post-termination ribosomal complexes that are devoid of the nascent peptide. PMID: 28899899
    2. UPF3B gene mutation is associated with Lujan-Fryns syndrome. PMID: 26358559
    3. the neurodevelopmental phenotype of UPF3B missense mutation is caused by impairment of nonsense-mediated mRNA decay pathway function altering neuronal differentiation. PMID: 26012578
    4. These findings indicate that SATB2 activates UPF3B expression through binding to its promoter. PMID: 23925499
    5. Data indicate the mutation p.R430X of UPF3B gene as the genetic etiology in the mental retardation pedigree. PMID: 22957832
    6. results demonstrate that the UPF3B-dependent NMD pathway is a major regulator of the transcriptome and that its targets have important roles in neuronal cells. PMID: 22182939
    7. The two cases with renal dysplasia and developmental delay showed remarkable clinical variability despite having the same mutation in UPF3B. PMID: 22609145
    8. Our results demonstrate that in addition to Lujan-Fryns and FG syndromes, UPF3B protein truncation mutations can cause also nonspecific XLMR. PMID: 19238151
    9. 3.4 A resolution crystal structure of a minimal UPF3b-EJC assembly, consisting of the interacting domains of five proteins (UPF3b, MAGO, Y14, eIF4AIII, and Barentsz) together with RNA and adenylyl-imidodiphosphate PMID: 20479275
    10. A conserved domain of hUpf3b mediates an interaction with the EJC protein Y14. Y14 is required for nonsense-mediated decay induced by tethered hUpf3b. PMID: 12718880
    11. The protein region that mediates this interaction and discriminates between hUpf3a and hUpf3b in NMD function is located in the C-terminal domain and fully contained within a small sequence that is highly conserved in Upf3b but not Upf3a proteins PMID: 16601204
    12. UPF3B induces nonsense mediated decay in the cytoplasm PMID: 17194930
    13. Three mutations lead to the introduction of a premature termination codon and subsequent nonsense-mediated mRNA decay of mutant UPF3B mRNA. PMID: 17704778
    14. UPF2 and UPF3b cooperatively stimulate both ATPase and RNA helicase activities of UPF1. PMID: 18066079
    15. Results suggest that UPF3A levels are tightly regulated by a post-transcriptional switch to maintain appropriate levels of NMD substrates in cells containing different levels of UPF3B. PMID: 19503078
    16. binds to spliced mRNAs upstream of exon-exon junctions; is part of mRNP complexes that are ready for nuclear export and that participate in nonsense-mediated mRNA decay PMID: 11546873
    17. binds RNPS1 protein, part of the postsplicing complex deposited 5' to exon-exon junctions PMID: 11546874

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  • 相关疾病：
    Mental retardation, X-linked, syndromic, 14 (MRXS14)
  • 亚细胞定位：
    Nucleus. Cytoplasm. Note=Shuttling between the nucleus and the cytoplasm.
  • 蛋白家族：
    RENT3 family
  • 组织特异性：
    Expressed in testis, uterus, prostate, heart, muscle, brain, spinal cord and placenta.
  • 数据库链接：

    HGNC: 20439

    OMIM: 300298

    KEGG: hsa:65109

    STRING: 9606.ENSP00000276201

    UniGene: Hs.103832