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UBE3B Antibody

  • 货号：
    CSB-PA070196
  • 规格：
    ￥880
  • 其他：

产品详情

  • Uniprot No.：
    Q7Z3V4
  • 基因名：
  • 别名：
    UBE3B antibody; Ubiquitin-protein ligase E3B antibody; EC 2.3.2.26 antibody; HECT-type ubiquitin transferase E3B antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human UBE3B.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, IHC, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    ELISA 1:10000
  • 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：
    E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfers the ubiquitin to targeted substrates.
  • 基因功能参考文献：
    1. we present four patients with five novel UBE3B mutations and propose the inclusion of clinical features to the characteristics of Kaufman oculocerebrofacial syndrome, including prominence of the cheeks and limb anomalies. PMID: 29160006
    2. studies demonstrate that UBE3B is an E3 ubiquitin ligase and reveal that the enzyme is regulated by calmodulin. Furthermore, the modulation of UBE3B via calmodulin and calcium implicates a role for calcium signaling in mitochondrial protein ubiquitylation, protein turnover, and disease PMID: 28003368
    3. Sanger sequencing was negative for the DOORS syndrome gene TBC1D24 but exome sequencing identified a homozygous deletion in UBE3B (NM_183415:c.3139_3141del, p.1047_1047del) located within the terminal portion of the HECT domain PMID: 28003643
    4. The E3 ligase activity of UBE3B is regulated by its interaction with calmodulin via the N-terminal IQ domain. PMID: 28003368
    5. UBE3B encodes a widely expressed protein ubiquitin ligase E3B, which, when mutated in both alleles, causes Kaufman oculocerebrofacial syndrome. PMID: 25691420
    6. UBE3B mutations cause a clinically recognizable and possibly underdiagnosed syndrome named the Kaufman oculocerebrofacial syndrome PMID: 24615390
    7. data provide evidence that Kaufman oculocerebrofacial syndrome is caused by UBE3B loss of function, and further demonstrate the impact of misregulation of protein ubiquitination on development and growth. PMID: 23687348
    8. Our data reveal the pleiotropic effects of UBE3B deficiency and reinforce the physiological importance of ubiquitination in neuronal development and function in mammals. PMID: 23200864
    9. UBE3B is a novel E3 ligase, with a HECT-domain which constitutes the active site for ubiquitin transfer PMID: 12837265
    10. the apparent occurrence of an unusual TG 3' splice site in intron 25 is discussed PMID: 17672918

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  • 相关疾病：
    Kaufman oculocerebrofacial syndrome (KOS)
  • 组织特异性：
    Widely expressed.
  • 数据库链接：

    HGNC: 13478

    OMIM: 244450

    KEGG: hsa:89910

    STRING: 9606.ENSP00000340596

    UniGene: Hs.374067