博奥森.bs-0586R R血管假性血友病因子/血管性血友病因子抗体 50ulBioss-耗材-金山科研平台 Thermo Fisher官网Nunc、QSP、Nalgene、Invitrogen、Gibco耗材试剂授权一级代理

博奥森.bs-0586R R血管假性血友病因子/血管性血友病因子抗体 50ulBioss

货品编号：博奥森.bs-0586R

品牌：博奥森

品名： R血管假性血友病因子/血管性血友病因子抗体

规格： 50ul

产品介绍

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.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.

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博奥森.bs-0586R R血管假性血友病因子/血管性血友病因子抗体 50ulBioss

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