von Willebrand antigen 2,血管性血友病因子抗体

产品编号HZ-4754R
英文名称von Willebrand antigen 2
中文名称血管假性血友病因子/血管性血友病因子抗体
别 名Von Willebrand Factor; Coagulation factor VIII; F8VWF; Factor VIII related antigen; von Willebrand antigen 2; Von Willebrand antigen II; Von Willebrand disease; VWD; VWF; VWF_HUMAN.
说 明 书0.1ml 0.2ml
研究领域心血管 细胞生物 免疫学 干细胞 血管内皮细胞
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Rabbit,
von Willebrand antigen 2,血管性血友病因子抗体产品应用IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量81kDa
细胞定位细胞外基质 分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human von Willebrand antigen 2
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
von Willebrand antigen 2,血管性血友病因子抗体PubMedPubMed
产品介绍background:
Von Willebrand Factor (VWF) was previously known as Factor VIII related antigen. VWF is synthesized exclusively by endothelial cells and megakaryocytes, and stored in the intracellular granules or constitutively secreted into plasma. This glycoprotein functions as both an antihemophilic factor carrier and a platelet vessel wall mediator in the blood coagulation system. Important in the maintenance of homeostasis, it participates in platelet vessel wall interactions by forming a noncovalent complex with coagulation factor VIII at the site of vascular injury. The Von Willebrand factor has functional binding domains to platelet glycoprotein Ib, glycoprotein IIb/IIIa, collagen and heparin. Mutations in this gene or deficiencies in this protein result in Von Willebrand's disease. VWD is characterized by frequent bleeding (gingival, minor skin quantitative lacerations, menorrhagia, etc.).

Function:
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.

Subunit:
Multimeric. Interacts with F8.

Subcellular Location:
Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules.

Tissue Specificity:
Plasma.

von Willebrand antigen 2,血管性血友病因子抗体Post-translational modifications:
All cysteine residues are involved in intrachain or interchain disulfide bonds.
N- and O-glycosylated.

DISEASE:
Defects in VWF are the cause of von Willebrand disease type 1 (VWD1) [MIM:193400]. A common hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 1 is characterized by partial quantitative deficiency of circulating von Willebrand factor, that is otherwise structurally and functionally normal. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma.
Defects in VWF are the cause of von Willebrand disease type 2 (VWD2) [MIM:613554]. A hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 2 is characterized by qualitative deficiency and functional anomalies of von Willebrand factor. It is divided in different subtypes including 2A, 2B, 2M and 2N (Normandy variant). The mutant VWF protein in types 2A, 2B and 2M are defective in their platelet-dependent function, whereas the mutant protein in type 2N is defective in its ability to bind factor VIII. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma.
Defects in VWF are the cause of von Willebrand disease type 3 (VWD3) [MIM:277480]. A severe hemorrhagic disorder due to a total or near total absence of von Willebrand factor in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII. Bleeding usually starts in infancy and can include epistaxis, recurrent mucocutaneous bleeding, excessive bleeding after minor trauma, and hemarthroses.

Similarity:
Contains 1 CTCK (C-terminal cystine knot-like) domain.
Contains 4 TIL (trypsin inhibitory-like) domains.
Contains 3 VWFA domains.
Contains 3 VWFC domains.
Contains 4 VWFD domains.

Gene ID:
7450

Database links:
Entrez Gene: 280958 Cow
Entrez Gene: 399544 Dog
Entrez Gene: 7450 Human
Entrez Gene: 399543 Pig
Entrez Gene: 116669 Rat
Omim: 613160 Human
SwissProt: Q28295 Dog
SwissProt: P04275 Human
SwissProt: Q28833 Pig
SwissProt: Q62935 Rat
Unigene: 440848 Human
Unigene: 35561 Rat

von Willebrand antigen 2,血管性血友病因子抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

血管性血友病因子(vWF)是血管内皮细胞和骨髓巨核细胞合成的一种糖蛋白，在1期和2期止血中都起着重要作用，如缺乏将导致患者出现血管性血友病(vWD)。vWF可被ADAMTS13裂解以失去活性，血小板反应蛋白/凝血酶敏感蛋白-1(Thrombospondin,TSP-1))可参与了这个调节过程。vWF水平受多种遗传和环境因素影响，其中ABO血型影响较大。vWF主要通过A1和A3区与血小板GP 1b和胶原结合，在止血和血栓形成过程中起重要作用，并与心、脑血管疾病及血管新生密切相关，因此研究vWF的生物学特性和功能具有重要的意义。