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  4. Human von Willebrand Factor,vWF ELISA Kit

Human von Willebrand Factor,vWF ELISA Kit

  • 中文名称：
    人血管性血友病因子/瑞斯托霉素辅因子(VWF)酶联免疫试剂盒
  • 货号：
    CSB-E08437h
  • 规格：
    96T/48T
  • 价格：
    ￥3600/￥2500
  • 其他：

产品详情

  • 产品描述：

    The human VWF ELISA kit (CSB-E08437h) is designed for the quantitative measurement of human VWF protein in samples. It shows excellent specificity for human VWF. It uses the bi-antibody sandwich enzyme immunoassay technique. This assay employs a biotin-conjugated VWF antibody that recognizes the analyte bound by the immobilized VWF antibody, forming an antibody-analyte-antibody complex. The immune complex is further detected by avidin-conjugated HRP. The TMB solution is added into the wells and turns blue and finally turns yellow after the addition of the stop solution. Solution color develops in proportion to the amount of VWF in the sample. The O.D. value is measured using a microplate reader at 450 nm and is used to determine the concentration of the human VWF in the sample.

    VWF plays a dual role in hemostasis. It promotes platelet adhesion by anchoring the platelets to the subendothelial matrix of injured vessels and also protects FVIII from proteolytic degradation. Moreover, VWF acts as an acute-phase protein that has multiple roles in vascular inflammation and is massively secreted from Weibel-Palade bodies upon endothelial cell activation. VWF deficiency, either quantitative or qualitative, leads to von Willebrand disease (VWD), the most common bleeding disorder. The VWF/ADAMTS13 signaling is involved in the pathogenesis of atherosclerosis, facilitating plaque formation and inflammation through macrophage and neutrophil recruitment in inflamed lesions. Recently, VWF and ADAMTS13 have been proposed as prognostic biomarkers in cardiovascular, metabolic, and inflammatory diseases, such as diabetes, stroke, and sepsis.

  • 别名：
    Coagulation factor VIII ELISA Kit; Coagulation factor VIII VWF ELISA Kit; F8VWF ELISA Kit; Factor VIII related antigen ELISA Kit; von Willebrand antigen 2 ELISA Kit; von Willebrand antigen II ELISA Kit; Von Willebrand disease ELISA Kit; Von Willebrand factor precursor ELISA Kit; VWD ELISA Kit; vWF ELISA Kit; VWF_HUMAN ELISA Kit
  • 缩写：
  • Uniprot No.：
  • 种属：
    Homo sapiens (Human)
  • 样本类型：
    serum, plasma, tissue homogenates.
  • 检测范围：
    6.25 ng/ml-400 ng/ml.
  • 灵敏度：
    1.56 ng/ml.
  • 反应时间：
    1-5h
  • 样本体积：
    50-100ul
  • 检测波长：
    450 nm
  • 研究领域：
    Cardiovascular
  • 测定原理：
    quantitative
  • 测定方法：
    Sandwich
  • 精密度：

      

  • 线性度：

      

  • 回收率：

      

  • 标准曲线：

      

  • 数据处理：
  • 货期：
    3-5 working days

产品评价

问答及客户评论

■ 常见问题解答
Q:

I want to detect the factor VIII of VWF (von Willebrand factor).
Please confirm that the kit (CSB-E08437h) can measure factor VIII of VWF.
If yes, please provide your reference data for samples, your lab tested.
If not, could you please recommend a suitable kit for my purposes.
Please provide the current manual and the information about kit’s components:
a. Capture antibody or capture antigen.
b. Detection antibody (if applicable).
c. Standard – if recombinant protein, please specify sequence (including species source and accession no. if available) and expression host.

A:
Thanks for your inquiry.
CSB-E08437h
This kit is designed according to: https://www.uniprot.org/uniprot/P04275.
It detects VWF in human samples.
Capture antibody is mouse monoclonal antibody.
Detection antibody is goat polyclonal antibody.
Standard is NS0-derived recombinant protein.
I will send you the kit manual by email.
We tested serum and plasma samples before and the test value is 25--100ng/ml.

靶点详情

  • 功能：
    Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma.
  • 基因功能参考文献：
    1. High VWF expression is associated with age-related macular degeneration. PMID: 29367644
    2. The Plasma Levels of ADAMTS-13, von Willebrand Factor, VWFpp, and Fibrin-Related Markers in Patients With Systemic Sclerosis Having Thrombosis. PMID: 29130325
    3. vWF acts as a simple prognostic biomarker in AF and, whilst its addition to current scores statistically improves prediction for some endpoints, absolute changes and impact on clinical decision-making are marginal. PMID: 28134282
    4. The aim of the study was to investigate the role of von Willebrand factor (vWF), the vWF-cleaving protease, ADAMTS13, the composition of thrombus, and patient outcome following mechanical cerebral artery thrombectomy in patients with acute ischemic stroke. PMID: 29887594
    5. High vWF plasma concentrations may discriminate patients with non-valvular atrial fibrillation at greater risk of stroke or all-cause death. PMID: 29685722
    6. Coronary artery disease patients exhibited significantly lower plasma BDNF and higher vWF levels than those of control patients. PMID: 29409455
    7. A discontinuous autoinhibitory module masks the A1 domain of von Willebrand factor, preventing binding to GPIba. PMID: 28692141
    8. VWF, GMP-140, ADAMTS13 and the cerebral vasospasm, delayed cerebral ischemia, tumor diameter and prognosis of aneurysmal subarachnoid hemorrhage patients are closely related PMID: 29077161
    9. von Willebrand Factor is steadily elevated throughout the course of dysrhythmia in NVAF patients treated with warfarin and in those with higher intensity of left atrium blood stasis. PMID: 28952167
    10. The levels of VWF-cleaving protease ADAMTS13 among neonates were higher as compared with healthy adults, despite a significant elevation of VWF antigen (Ag) and Ristocetin cofactor (RiCof) noted in all neonates. PMID: 28087247
    11. The pathogenic effects of candidate VWF gene mutations were explored in this study. Molecular dynamic simulations on p.M771I mutant VWF revealed distinct structural rearrangements including a large deviation in the E' domain, and significant loss of beta-sheet secondary structure. PMID: 27483487
    12. investigated the roles of ADAMTS13 and VWF in thrombotic events of patients with Connective Tissue Diseases PMID: 26759371
    13. vWF had a significant prognostic impact on cardiovascular mortality when OPG levels were low. Low levels of both OPG and act vWF were associated with a 99 % survival rate during the follow-up of five years. PMID: 28726980
    14. Studied the significance of the von Willebrand factor (VWF)/ ADAMTS-13 ratio in advanced non-small-cell lung cancer (NSCLC). Findings suggest that the imbalance between VWF secretion and ADAMTS-13 may play a role in the hypercoagulability state in advanced NSCLC, and increase of the plasma VWF/ADAMTS-13 ratio may serve as an independent predictive factor for mortality in patients with advanced NSCLC. PMID: 28374895
    15. Type 3 von Willebrand disease patients with the Pro2808Leufs*24 have bioavailable platelet-derived VWF that may produce a milder bleeding phenotype than other type 3s PMID: 28453889
    16. vaso-occlusive crisis in sickle cell disease is associated with increased reactivity of VWF, without a pronounced ADAMTS-13 deficiency PMID: 28457019
    17. Overexpression of factor VIII-RA in the endothelial cells of capillary like vessels in the periphery of central and peripheral giant cell granuloma of jaw was prominent. PMID: 28832079
    18. Different patterns of VWF multimer loss were seen in heart failure patients treated with implantation of left ventricular assist device and total artificial heart, which may contribute to bleeding patterns. PMID: 28586149
    19. A high degree of clinical and laboratory variability exists within type 2M von Willebrand disease variants. PMID: 28544236
    20. Complex VWF-ADAMTS13-mediated mechanisms disturb haemostasis in inflammatory bowel disease. PMID: 28765701
    21. PP2B-Aalpha-Munc18c complex supports agonist-induced VWF secretion by HUVECs. PMID: 28294518
    22. Deficiency of the von Willebrand factor-cleaving protease ADAMTS13 is central to the pathophysiology of thrombotic thrombocytopenic purpura. Reviewed is the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of acute coronary syndrome. [review] PMID: 28521259
    23. genetic variation of vWF V1565L polymorphism modulates ADAMTS13 activity. PMID: 27536857
    24. Plasma levels of VWF antigen and activity are significantly increased with concomitant reduction in plasma ADAMTS13 activity in patients with subarachnoid haemorrhage. PMID: 28102428
    25. Report a diagnostic algorithm that can reliably identify pathogenic variants of factor 8/9 and von Willebrand factor and diagnose patients with hemophilia A, hemophilia B or von Willebrand disease. PMID: 27734074
    26. analysis of an artificial botrocetin that can inhibit the VWF-GPIb interaction PMID: 28071872
    27. VWF binding to MMRN1 was enhanced by shear exposure and ristocetin, and required VWF A1A2A3 region, specifically the A1 and A3 domains. PMID: 27052467
    28. coronary thrombi in STEMI patients resistant to fibrinolysis are characterised by higher fibrin, P-selectin and VWF content than lysis-sensitive thrombi PMID: 26962963
    29. We compared the classical Sanger sequencing approach and NGS to assess the value-added effect on the analysis of the mutation distribution in different types of VWD. Sixty-two different VWF mutations were identified, 27 of which had not been previously described. NGS detected 26 additional mutations, contributing to a broad overview of the mutant alleles present in each VWD type. PMID: 26988807
    30. These observations support the hypothesis that a significantly reduced ADAMTS13/VWF ratio in the coronary artery flow plays a pathogenic role in acute coronary syndromes (ACS) and suggest that transition from laminar to turbulent flow at sites of coronary stenosis further enhances VWF activation and deposition. PMID: 27034431
    31. the major genetic factors affecting plasma VWF levels, i.e. variants at ABO, VWF and a locus on chromosome 2, operate primarily through their effects on VWF clearance PMID: 27359253
    32. The unique phenotype of VWD2 type IIC-Miami results from dominant impairment of multimer assembly, an aberrant structure of mutant mature VWF and reduced clearance in vivo PMID: 27344059
    33. Low ADAMTS-13 activity is associated with an increased risk of coronary heart disease in the elderly, independently of VWF and established cardiovascular risk factors PMID: 27559008
    34. 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
    35. N-linked glycan truncation causes enhanced clearance of plasma-derived von Willebrand factor; variation in VWF glycosylation may be important in the pathophysiology underlying type 1C VWD PMID: 27732771
    36. 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
    37. In people with dysglycaemia and other cardiovascular risk factors, basal insulin does not improve the levels of markers of fibrinolysis or von Willebrand factor compared to standard glucose-lowering treatments. PMID: 28403644
    38. low VWF levels can be associated with significant bleeding and are predominantly due to reductions in VWF synthesis and/or constitutive secretion. PMID: 28916584
    39. Refrigeration-induced binding of VWF to platelets facilitates their rapid clearance by inducing GPIbalpha-mediated signaling. PMID: 29097365
    40. 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
    41. 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
    42. von Willebrand factor processing and function has been summarized using various biophysical techniques. (Review) PMID: 27717713
    43. Soluble VWF was statistically elevated in major depressive disorder. PMID: 27598970
    44. Rare genetic variants in the ADAMTS13 on Willebrand factor-binding domain contribute to pediatric stroke. PMID: 27412500
    45. results indicate that VWF multimer accumulation on endothelium, as occurs in congenital thrombotic thrombocytopenic purpura, promotes the activation of the complement alternative pathway that proceeds until C5 cleavage with the formation of C5a and the terminal C5b-9 complex PMID: 28652401
    46. Findings also identify VWF as a new complement regulator on vascular endothelial cells and suggest that VWF has a protective effect on endothelial cells and complement-mediated injury. PMID: 27236750
    47. LVAD recipients achieved a new hemostatic equilibrium characterized by infrequent major hemorrhagic and thrombotic events, despite a mildly impaired vWF function and a markedly enhanced thrombin formation. PMID: 27485105
    48. These results demonstrate that cancer cells of non-endothelial origin can acquire de novo expression of VWF, which can enhance processes, including endothelial and platelet adhesion and extravasation, that contribute to cancer metastasis. PMID: 28035064
    49. level of vWF-ag in the chronic phase correlated with the amounts of VAT and PAT, but not with subcutaneous adipose tissue PMID: 28570705
    50. both in acute and chronic cerebrovascular disease patients, ADAMTS13 levels were significantly decreased, with the lowest ADAMTS13 levels found in acute stroke patients. This difference was even more distinct when the ratio of VWF:ADAMTS13 was considered. These results demonstrate the potentially important involvement of the VWF/ADAMTS13 axis in ischemic stroke. PMID: 28591212

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  • 相关疾病：
    von Willebrand disease 1 (VWD1); von Willebrand disease 2 (VWD2); von Willebrand disease 3 (VWD3)
  • 亚细胞定位：
    Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules.
  • 组织特异性：
    Plasma.
  • 数据库链接：

    HGNC: 12726

    OMIM: 193400

    KEGG: hsa:7450

    STRING: 9606.ENSP00000261405

    UniGene: Hs.440848