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货号 | bs-10048R-1 |
品牌 | |
浓度 | |
货期 | 现货 |
英文名称 | Rabbit Anti-Von Willebrand Factor antibody |
中文名称 | Rabbit Anti-Von Willebrand Factor antibody |
研究领域 | 肿瘤,心血管,细胞生物,发育生物学,生长因子和激素 |
英文别名 | Von Willebrand Factor; Coagulation factor VIII; F8VWF; Factor VIII related antigen; von Willebrand antigen 2; Von Willebrand antigen II; Von Willebrand disease; VWD; Coagulation factor VIII; Coagulation factor VIII VWF; F8VWF; VWF_HUMAN. |
反应物种(已验证) | Human,Mouse,Rat |
反应物种(预测) | Dog,Cow,Horse,Rabbit |
产品应用(已验证) | IHC,IF,FCM |
推荐稀释比例 | IHC-P=1:100-500,IHC-F=1:100-500,Flow Cyt=3ug/Test,IF=1:100-500, |
克隆类型 | 多克隆 |
抗体来源 | Rabbit |
理论分子量 | 226/309 |
细胞定位 | 细胞外基质,分泌型蛋白 |
性状 | Liquid |
免疫原 | KLH conjugated synthetic peptide derived from human VWF/Von Willebrand Factor |
抗原表位 | 1651-1800/2813 |
亚型 | IgG |
纯化方法 | affinity purified by Protein A |
SUBCELLULAR | Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules. |
Tissue | Plasma. |
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. |
SUBUNIT | Multimeric. Interacts with F8. |
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 f |
Post-translational | 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 |
SWISS | P04275 |
Gene ID | 7450 |
保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 | 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.). |