protein

Human von Willebrand Factor, Factor VIII Free

MBS135992

0.1 mg

850 USD

Native Protein

Human von Willebrand Factor, Factor VIII Free

[von Willebrand Factor]

[von Willebrand factor preproprotein; von Willebrand factor; von Willebrand factor; coagulation factor VIII VWF; von Willebrand factor; von Willebrand antigen II]

[VWF; VWF; VWD; F8VWF; F8VWF; vWF]

VWF_HUMAN

Human

2813

< 1% Factor VIII Activity

>95% by SDS-PAGE analysis

Frozen liquid

0.18 mg/mL

Recommended Storage: At -70°C. Minimum Shelf Life: 3 years from delivery. Shipping Conditions: Dry ice

Source Info: Human plasma

Buffer: 0.025M Sodium Citrate; 0.1M NaCl; 0.1M glycine; pH 6.8

VON WILLEBRAND FACTOR

vWF is prepared from citrated human plasma using a combination of chromotographic procedures. Although the starting material was tested prior to initiation of the manufacturing process, and was found negative or nonreactive for anti-HIV-1/2, HIV-1 antigen(s), HBsAg, STS, anti-HCV, anti-HBcore and anti-HTLV I & II, extreme caution should be used when handling this material as there is a margin of error in all tests. This preparation is >95percent pure as judged by SDS-PAGE under reducing conditions, and consists of large multimers as determined by electrophoresis in SDS/agarose gels. Molecular weight under native conditions ranges from 520 kDa (dimer) to >10,000 kDa (multimers). Protein concentration determined by total protein assay. Required thawing procedure: Remove the sample from freezer and place in a 37 degree celsius water bath. Allow the vial to remain in the water bath for 2 minutes following complete disappearance of the frozen material. Remove sample and gently mix with a pipet or by gentle agitation. If precipitate is observed, place in water bath for an additional 2-3 minutes then mix again.

89191868

NP_000543.2

NM_000552.3

P04275

260,000 (Monomer)

Blood Clotting Cascade Pathway (198840); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Focal Adhesion Pathway (198795); Focal Adhesion Pathway (83067); Focal Adhesion Pathway (478); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057)

The glycoprotein encoded by this gene functions as both an antihemophilic factor carrier and a platelet-vessel wall mediator in the blood coagulation system. It is crucial to the hemostasis process. Mutations in this gene or deficiencies in this protein result in von Willebrand's disease. An unprocessed pseudogene has been found on chromosome 22. [provided by RefSeq, Jul 2008]

VWF: 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. Defects in VWF are the cause of von Willebrand disease type 1 (VWD1). 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). 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). 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. Protein type: Extracellular matrix; Motility/polarity/chemotaxis; Secreted, signal peptide; Cell adhesion; Secreted. Chromosomal Location of Human Ortholog: 12p13.3. Cellular Component: extracellular matrix; proteinaceous extracellular matrix; endoplasmic reticulum; extracellular region; external side of plasma membrane. Molecular Function: integrin binding; collagen binding; identical protein binding; protein binding; protein homodimerization activity; protease binding; chaperone binding; immunoglobulin binding; protein N-terminus binding; glycoprotein binding. Biological Process: platelet activation; extracellular matrix organization and biogenesis; platelet degranulation; hemostasis; response to wounding; cell adhesion; liver development; blood coagulation; blood coagulation, intrinsic pathway; protein homooligomerization; cell-substrate adhesion; placenta development. Disease: Von Willebrand Disease, Type 3; Von Willebrand Disease, Type 1; Von Willebrand Disease, Type 2

0.1 mg

850 USD

0.5 mg

2690