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  4. Recombinant Human Platelet glycoprotein Ib alpha chain (GP1BA), partial

Recombinant Human Platelet glycoprotein Ib alpha chain (GP1BA), partial

  • 货号：
    CSB-EP009685HU1
  • 规格：
    ￥1836
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 85% as determined by SDS-PAGE.
  • 基因名：
  • Uniprot No.：
  • 别名：
    GP1BA; Platelet glycoprotein Ib alpha chain; GP-Ib alpha; GPIb-alpha; GPIbA; Glycoprotein Ibalpha; Antigen CD42b-alpha; CD antigen CD42b) [Cleaved into: Glycocalicin]
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Partial
  • 来源：
    E.coli
  • 分子量：
    15.9 kDa
  • 表达区域：
    553-652aa
  • 氨基酸序列
    SWVGHVKPQALDSGQGAALTTATQTTHLELQRGRQVTVPRAWLLFLRGSLPTFRSSLFLWVRPNGRVGPLVAGRRPSALSQGRGQDLLSTVSIRYSGHSL
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 10xHis-tagged and C-terminal Myc-tagged
  • 产品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

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 the A1 domain of vWF, which is already bound to the subendothelium.
  • 基因功能参考文献：
    1. An autosomal dominant mode of inheritance, a family history of mild bleeding episodes, aggregation pattern in affected individuals together with evidence of mutation occurring in part of the GP1BA gene encoding the leucine-rich repeat region suggest a novel variant causing monoallelic Bernard-Soulier syndrome. PMID: 30332551
    2. Combined deficiency of factors V and VIII by chance coinheritance of parahaemophilia and haemophilia A, but not by mutations of either LMAN1 or MCFD2 PMID: 29119711
    3. A review of mutations associated with Bernard-Soulier Syndrome and platelet type von Willebrand disease (review). PMID: 28961024
    4. ERK5 associates with CKII to play essential roles in GPIb-IX-mediated platelet activation via the PTEN/PI3K/Akt pathway. PMID: 28603902
    5. There was no evidence to suggest that polymorphisms of GP VI T13254C and GP Ibalpha VNTR were associated with CAD. PMID: 28607925
    6. analysis of an artificial botrocetin that can inhibit the VWF-GPIb interaction PMID: 28071872
    7. Loss of the platelet surface receptors GPIbalpha and GPVI in heart failure, CF-VAD and ECMO patients may contribute to ablated platelet adhesion/activation, and limit thrombus formation under high/pathologic shear conditions PMID: 27601054
    8. Very low birth weight preterm neonates have increased numbers of platelets interacting with von Willebrand Factor, and increased GPIbalpha expression on the platelet surface PMID: 27416003
    9. Data suggest that an aspartate at position 1261 is the most critical residue of VWF N-terminal linker for inhibiting binding of VWF A1 domain to GP1BA on platelets in a model simulating blood flow velocity; network of salt bridges between Asp1261 and rest of VWF A1 domain lock N-terminal linker in place such that binding to GP1BA is reduced. PMID: 28924049
    10. Data show that von Willebrand factor (VWF) is first converted from a compact to linear form by flow, and is subsequently activated to bind platelet glycoprotein Ib alpha polypeptide (GPIbalpha) in a tension-dependent manner. PMID: 28831047
    11. The >30 nm macroglycopeptide separating the two domains of GPIbalpha transmits force on the VWF-GPIbalpha bond (whose lifetime is prolonged by leucine-rich repeat domain unfolding) to the juxtamembrane mechanosensitive domain to enhance its unfolding, resulting in unfolding cooperativity at an optimal force. PMID: 27434669
    12. Meta-analysis found that glycoprotein Ia C807T T allele or the TT genotype, the Ser-allele of HPA-3 and B allele of glycoprotein Ibalpha variable number tandem repeat polymorphisms were associated with increased risk for ischemic stroke. PMID: 28004990
    13. Our results suggest that the -5CC genotype in Kozak sequence of GPIb-alpha may be associated with a higher risk of developing arterial ischemia of lower limbs in type 2 diabetes mellitus patients. PMID: 27888791
    14. Specific inhibition of GPIbalpha shedding in the stored platelets improves post-transfusion platelet recovery and hemostatic function, providing clear evidence for GPIbalpha shedding as a cause of platelet clearance. PMID: 27417583
    15. miR-10a and miR10b regulate the expression of human platelet GP1BA and GP1bb for normal megakaryopoiesis. PMID: 27834869
    16. Data indicate that binding of hemoblobin (Hb) to glycoprotein1balpha (GP1balpha) induced platelet activation plays a crucial role in thrombus formation on immobilized von Willebrand factor (VWF) or type I collagen under shear stresses. PMID: 27105433
    17. Lateral dimerization of GPIbalpha induced by antibody binding is not sufficient to initiate GPIb-IX signaling and induce platelet clearance. PMID: 26662889
    18. GPIb alpha plays a critical role in the co-localization of thrombin and factor XI and the resultant efficient activation of FXI PMID: 12968031
    19. Hemoglobin interaction with GP1balpha induces platelet activation and apoptosis: a novel mechanism associated with intravascular hemolysis. PMID: 26341739
    20. Both GPIbalpha and PAR4 are required for thrombin-induced reactive oxygen species formation PMID: 26569550
    21. Our results reveal the molecular mechanism of collagen-regulated, A1-mediated platelet adhesion enhancement. PMID: 26213126
    22. Report no relationship, between polymorphisms of platelet membrane glycoprotein Ibalpha and risk of coronary heart disease in Chinese Han population. PMID: 26191334
    23. There is a prevalence of Thr145Met and T(-5)C GP Iba polymorphiams in patients with atherotrombotic stroke due to macroangiopathy. PMID: 26539867
    24. Molecular analysis demonstrated a novel homozygous c.800C>G substitution in GP1BA exon 2 leading to a serine 267 Ter stop codon in all 3 siblings PMID: 26044173
    25. Whereas VWF-D'D3 is the major regulator of soluble VWF binding to platelet GpIbalpha, both the D'D3-domain and N-terminal peptide regulate platelet translocation and thrombus formation. PMID: 25341886
    26. Data indicate that GPIbalpha clustering induced by anti-GPIbalpha N-terminus antibody causes integrin alphaIIbbeta3-dependent platelet aggregation, phagocytosis, and rapid platelet clearance in the liver. PMID: 25231551
    27. Data show that force can switch the kinetics of bond formation between A1 domain of von Willebrand factor (VWF) and glycoprotein Ibalpha (GPIbalpha). PMID: 25810255
    28. novel mutation that causes von Willebrand disease type 2B is identified in a German family PMID: 24337418
    29. The platelet adhesion receptor, the glycoprotein Ib-IX-V complex, not only mediates platelet adhesion but also transmits signals leading to platelet activation, aggregation and secretion PMID: 17414217
    30. data indicated that GPIIb-IIIa and GPIb levels are mainly affected by platelet size (MPV) but not by their genetic variations; in some acute coronary syndrome patients, production of large platelets with high GPIIb-IIIa and GPIb contents might be stimulated by elevated thrombopoietin PMID: 23941967
    31. Data indicate a G > T in platelet glycoprotein Ib alpha polypeptide (GP1BA) gene, resulting in a Trp to Leu amino acid change at residue 246 (p.W246L), and this mutation was absent in his unaffected mother and also in the 100 controls. PMID: 24474090
    32. clone 5G6 showed similar inhibitory potency as a widely used shedding inhibitor GM6001 in both constitutive and induced GPIbalpha shedding in human platelets PMID: 24119228
    33. VWF interaction with glycoprotein Ib is modified by polyphosphate PMID: 23006049
    34. Our findings indicate that ADAM17 may be a risk factor for ischemic stroke in Chinese and the expression of GPIbalpha can serve as a measure for stroke severity. PMID: 23771674
    35. Platelet GP Ib-IX can be considered a multifunctional participant in hemostasis, thrombosis, and the inflammatory cascade. PMID: 24504734
    36. Increased mean platelet volume values correlated with increased platelet aggregation activity and enhanced GP IIb-IIIa and GP Ib expression. PMID: 24749250
    37. These findings suggest that structural changes, including central GPIbalpha LRR-A1 contact, contribute to VWF affinity regulation. PMID: 24391089
    38. genetic association study in population in western India: Data suggest novel mutations in platelet glycoprotein Ib (GP1BA, GP1BB) and GP9 are associated with Bernard-Soulier syndrome in subjects studies; of 12 mutations identified, ten were novel. PMID: 23995613
    39. Studies indicate that the platelet-type von Willebrand disease (PT-VWD) is caused by gain-of-function mutations in the platelet GP1BA gene, which codes for the platelet von Willebrand factor (VWF) receptor, GPIbalpha. PMID: 23934752
    40. Studies indicate that platelets from Bernard-Soulier syndrome (BSS) are defective in glycoprotein (GP)Ib-IX-V, a platelet-specific adhesion-signaling complex, composed of GPIbalpha disulfide linked to GPIbbeta, and noncovalently associated with GPIX and GPV. PMID: 23929303
    41. signaling process through the GPIbalpha cytoplasmic tail required for full platelet activation is defective in BSS variant case II and a length polymorphism of GPIbalpha is associated with a modified level of RIPA heterozygous BSS case I. PMID: 23414566
    42. analysis of the localized dynamics-driven affinity regulation mechanism for vWF-GPIbalpha interaction PMID: 23902764
    43. These data indicate an important role for the platelet adhesion receptor GPIb-IX in endotoxin-induced thrombosis and thrombocytopenia. PMID: 24051142
    44. A considerable fraction of the Jordanian population is resistant to the antiplatelet effect of aspirin, which might be related to GPIba C-5T polymorphism. PMID: 23688555
    45. A GP1BA exon 2 fragment spanned the HPA-2 polymorphism and adjacent sequences. PMID: 23750933
    46. genotype may predict the development of septic emboli in patients with infective endocarditis PMID: 23611001
    47. identify a novel Asp235Tyrmutation in the GP1BA gene of two Iranian patients showing the PT-VWD phenotype who were originally misdiagnosed as type 2B VWD PMID: 23014764
    48. GPIX increased the expression of GPIba by promoting the formation of a disulfide bond between GPIba and GPIbb in transfected CHO-K1 cells. PMID: 23143686
    49. Data indicate that exposure of von Willebrand factor sites for glycoprotein Ibalpha binding and ADAMTS13 cleavage are coupled. PMID: 22922961
    50. The T1255A, Clus1, and DC variants caused increased ristocetin-mediated GPIbalpha binding to VWF. PMID: 22517896

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  • 相关疾病：
    Non-arteritic anterior ischemic optic neuropathy (NAION); Bernard-Soulier syndrome (BSS); Bernard-Soulier syndrome A2, autosomal dominant (BSSA2); Pseudo-von Willebrand disease (VWDP)
  • 亚细胞定位：
    Membrane; Single-pass type I membrane protein.
  • 数据库链接：

    HGNC: 4439

    OMIM: 153670

    KEGG: hsa:2811

    STRING: 9606.ENSP00000329380

    UniGene: Hs.1472