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GP1BB Antibody

  • 货号：
    CSB-PA009686ESR1HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA009686ESR1HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA009686ESR1HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) GP1BB Polyclonal antibody
  • Uniprot No.：
    P13224
  • 基因名：
  • 别名：
    GP1BB; Platelet glycoprotein Ib beta chain; GP-Ib beta; GPIb-beta; GPIbB; Antigen CD42b-beta; CD antigen CD42c
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Platelet glycoprotein Ib beta chain protein (27-147AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 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：
    Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.
  • 基因功能参考文献：
    1. Rare variants in GP1BB are responsible for autosomal dominant macrothrombocytopenia. PMID: 28064200
    2. Data show that localization of the GP Ib-IX complex to the lipid domain is mediated by GP Ibbeta and GP IX transmembrane domains. PMID: 26203189
    3. Low levels of CD9 coincidental with a novel nonsense mutation in glycoprotein Ibbeta in a patient with Bernard-Soulier syndrome. PMID: 26275786
    4. a suspicion of 22q11.2 deletion is warranted in pediatric BSS patients with a mutation in the GPIbbeta gene, even without remarkable symptoms. PMID: 23566026
    5. GPIbbeta missense mutations from Bernard-Soulier syndrome were examined for changes to GPIb-IX complex surface expression. Mutations A108P and P74R were found to maintain normal secretion/folding of GPIbbeta(E) but were unable to support GPIX surface expression PMID: 21908432
    6. A novel variant of Bernard Soulier syndrome is described in which Ser23 of GPIbbeta is substituted by a Stop codon causing a premature termination of translation. PMID: 19484238
    7. A 14-month-old boy with Bernard-Soulier syndrome was found to be homozygous for a nonsense mutation (c.423C > A) in the glycoprotein Ib-beta. PMID: 22343686
    8. Identify TRAF4 as a novel binding partner for GPIb-IX-V and GPVI in human platelets. PMID: 20946164
    9. putative convex surface of the LRR domain in GPIX is sufficient, in the context of full-length subunit, to mediate its association with GPIbbeta PMID: 19566547
    10. Lateral clustering of platelet GP Ib-IX complexes leads to up-regulation of the adhesive function of integrin alpha IIbbeta 3 PMID: 11812775
    11. Role of GPIbbeta in modulating vWF mediated platelet adhesion. PMID: 11816713
    12. A GP1BB missense mutation suppresses GPIb/IX complex expression and is found to cause Bernard-Soulier Syndrome in the homozygous form and giant platelets in the heterozygous form. PMID: 11816714
    13. The cysteine knot of platelet GPIb beta is critical for the interaction of GPIb beta with GPIX. PMID: 12036872
    14. PKA-mediated phosphorylation of GPIbbeta at Ser(166) negatively regulates VWF binding to GPIb-IX and is one of the mechanisms by which PKA mediates platelet inhibition PMID: 12361948
    15. The GPIb beta intracellular domain has a role in controlling the adhesive properties of the GPIb/V/IX complex through phosphorylation of GPIb beta Ser166 and point to the existence of cross-talk between the GPIb beta and GPIb alpha intracellular domains. PMID: 12522011
    16. novel hemizygous variant of Bernard-Soulier Syndrome in which Pro29 in one GPIbbeta allele is substituted by a Leu PMID: 12529755
    17. To determine the sequence in the beta3 cytoplasmic domain that is critical to integrin signaling, cell lines were established that coexpress the platelet receptor for GP1BB. PMID: 12860973
    18. identified novel GPIbbeta mutation is responsible for the Bernard-Soulier syndrome phenotype and provide an explanation for the molecular mechanism underlying the reduced platelet content of GPIb-IX complex in the heterozygous individuals studied PMID: 12958615
    19. The SEPT5 gene resides approximately 250 nucleotides 5' to the GP Ibbeta gene and has been associated with modulating exocytosis from neurons and platelets as part of a presynaptic protein complex PMID: 15213102
    20. Velocardiofacial syndrome patients have in-vitro defects in platelet function that may increase their risk of bleeding during surgery. PMID: 18064328
    21. the N1421K substitution in the VWF affects the GPIb binding site or a recognition element by a conformational change of the A1 domain. PMID: 18637125
    22. proplatelet formation in human megakaryocytes undergoes a complex spatio-temporal regulation orchestrated by adhesive proteins, GPIb-IX-V and myosin IIA PMID: 18752571
    23. novel Ser 23 Stop mutation in GPIbbeta is responsible of BSS in the studied family and hampers the complex to form on the platelets surface. PMID: 18825380

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  • 相关疾病：
    Bernard-Soulier syndrome (BSS)
  • 亚细胞定位：
    Membrane; Single-pass type I membrane protein.
  • 组织特异性：
    Expressed in heart and brain.
  • 数据库链接：

    HGNC: 4440

    OMIM: 138720

    KEGG: hsa:2812

    STRING: 9606.ENSP00000383382

    UniGene: Hs.283743