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RBCK1 Antibody

  • 货号：
    CSB-PA880138LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human thyroid tissue using CSB-PA880138LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) RBCK1 Polyclonal antibody
  • Uniprot No.：
    Q9BYM8
  • 基因名：
  • 别名：
    C20orf18 antibody; Chromosome 20 open reading frame 18 antibody; HBV associated factor 4 antibody; HBV-associated factor 4 antibody; Heme-oxidized IRP2 ubiquitin ligase 1 antibody; Hepatitis B virus X associated protein 4 antibody; Hepatitis B virus X-associated protein 4 antibody; HOIL 1L antibody; HOIL-1 antibody; HOIL-1L antibody; HOIL1 antibody; HOIL1L antibody; RanBP type and C3HC4 type zinc finger containing 1 antibody; RanBP-type and C3HC4-type zinc finger-containing protein 1 antibody; RBCC protein interacting with PKC1 antibody; Rbck1 antibody; RBCK2 antibody; RING finger protein 54 antibody; RNF54 antibody; UB7I3_HUMAN antibody; UBCE7IP3 antibody; Ubiquitin conjugating enzyme 7 interacting protein 3 antibody; Ubiquitin-conjugating enzyme 7-interacting protein 3 antibody; XAP3 antibody; XAP4 antibody; ZRANB4 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human RanBP-type and C3HC4-type zinc finger-containing protein 1 protein (162-261AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，RBCK1 Antibody (CSB-PA880138LA01HU)，的标记方式是Non-conjugated。对于RBCK1 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-PA880138LB01HU RBCK1 Antibody, HRP conjugated ELISA
    FITC CSB-PA880138LC01HU RBCK1 Antibody, FITC conjugated
    Biotin CSB-PA880138LD01HU RBCK1 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
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • 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：
    E3 ubiquitin-protein ligase, which accepts ubiquitin from specific E2 ubiquitin-conjugating enzymes, such as UBE2L3/UBCM4, and then transfers it to substrates. Functions as an E3 ligase for oxidized IREB2 and both heme and oxygen are necessary for IREB2 ubiquitination. Promotes ubiquitination of TAB2 and IRF3 and their degradation by the proteasome. Component of the LUBAC complex which conjugates linear ('Met-1'-linked) polyubiquitin chains to substrates and plays a key role in NF-kappa-B activation and regulation of inflammation. LUBAC conjugates linear polyubiquitin to IKBKG and RIPK1 and is involved in activation of the canonical NF-kappa-B and the JNK signaling pathways. Linear ubiquitination mediated by the LUBAC complex interferes with TNF-induced cell death and thereby prevents inflammation. LUBAC is recruited to the TNF-R1 signaling complex (TNF-RSC) following polyubiquitination of TNF-RSC components by BIRC2 and/or BIRC3 and to conjugate linear polyubiquitin to IKBKG and possibly other components contributing to the stability of the complex. Together with OTULIN, the LUBAC complex regulates the canonical Wnt signaling during angiogenesis. Binds polyubiquitin of different linkage types.
  • 基因功能参考文献：
    1. This study demonstrated that Mutations outside the N-terminal part of RBCK1 may cause polyglucosan body myopathy with immunological dysfunction. PMID: 29260357
    2. The binding of SHARPIN or HOIL-1L facilitates the E2 loading of HOIP. PMID: 28978479
    3. LUBAC components control TLR3-mediated innate immunity, thereby preventing development of immunodeficiency and autoinflammation. PMID: 27810922
    4. results unveil HOIL1 as a negative regulator of lymphocyte activation cleaved by MALT1. PMID: 27006117
    5. HOXA1-mediated activation of NF-kappaB is non-transcriptional and the RBCK1 and TRAF2 influences on NF-kappaB are epistatic to HOXA1 PMID: 27382069
    6. Late in the NF-kappaB activation cycle HOIL1 cleavage transiently reduces linear ubiquitination, including of NEMO and RIP1, dampening NF-kappaB activation and preventing reactivation. PMID: 26525107
    7. human HOIP is essential for the assembly and function of LUBAC, which includes HOIL-1, and for various processes governing inflammation and immunity in both hematopoietic and nonhematopoietic cells PMID: 26008899
    8. findings support a role for RBCK1 in the regulation of FKBPL with important implications for estrogen receptor signaling, cell proliferation and response to endocrine therapy. PMID: 23912458
    9. RBCK1 deficiency is a frequent cause of polyglucosan storage myopathy associated with progressive muscle weakness and cardiomyopathy. PMID: 23798481
    10. Suggest that RBCK1 is important for the ubiquitination of PXR and may play a role in its proteasomal degradation. PMID: 23160820
    11. Estrogen receptor-alpha, RBCK1, and protein kinase C beta 1 cooperate to regulate estrogen receptor-alpha gene expression. PMID: 23042805
    12. Patients from two kindreds of a new fatal inherited disorder characterized by chronic autoinflammation, invasive bacterial infections and muscular amylopectinosis carried biallelic loss-of-expression and loss-of-function mutations in HOIL1. PMID: 23104095
    13. The solution structure of the HOIL1 Ubl domain was solved using NMR spectroscopy to compare it with that of parkin to determine the structural elements responsible for S5a subunit of the 26S proteasome intermolecular interactions. PMID: 22517668
    14. analysis of recognition of linear ubiquitin chains by the Npl4 zinc finger (NZF) domain of the HOIL-1L subunit of the linear ubiquitin chain assembly complex PMID: 22139374
    15. Data report the identification of the related proteins Sipl1 (Shank-interacting protein-like 1) and Rbck1 (RBCC protein interacting with PKC1) as novel interaction partners of Eya1. PMID: 20956555
    16. Findings suggest that RBCK1 regulates cell cycle progression and proliferation of ERalpha-positive breast cancer cells by supporting transcription of ERalpha and cyclin B1. PMID: 20103625
    17. RBCK1 (human C20orf18 and rat Pkcbpb15) is involved in transcriptional machinery in the nuclear bodies, and its transcriptional activity is regulated by nucleocytoplasmic shuttling. PMID: 15833741
    18. RBCK2 serves as a cytoplasmic tethering protein for RBCK1 PMID: 16083853
    19. HOIL-1 expression stabilizes SOCS6 and induces the ubiquitination and degradation of proteins associated with SOCS6 PMID: 16643902
    20. findings suggest that RBCK1 is involved in negative regulation of inflammatory signaling triggered by TNF and IL-1 through targeting TAB2/3 for degradation PMID: 17449468
    21. These data demonstrate that HOIL-1 is not required for iron-dependent degradation of IRP2 in HEK293 cells, and suggest that a HOIL-1 independent mechanism is used for IRP2 degradation in most cell types. PMID: 17822790
    22. the E3 activity of RBCK1 is controlled by two distinct manners, interaction with RBCK2 and phosphorylation by PKCbeta PMID: 18303026
    23. Study shows that the E3 ubiquitin ligase RBCC protein interacting with PKC1 (RBCK1) catalyzes the ubiquitination and degradation of IRF3. PMID: 18711448
    24. The shRNA barcode screening technique identified RBCK1 as being involved in p53 regulation. PMID: 19277210
    25. RBCK2 functions as an adaptor protein for the polyubiquitinated protein and the S5a subunit in 26S proteasome through its novel zinc finger motif and ubiquitin-like domain, respectively. PMID: 19796170

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  • 相关疾病：
    Polyglucosan body myopathy 1 with or without immunodeficiency (PGBM1)
  • 数据库链接：

    HGNC: 15864

    OMIM: 610924

    KEGG: hsa:10616

    STRING: 9606.ENSP00000348632

    UniGene: Hs.247280