Cardiovascular Antibodies

Anti-VWF Antibody (CAB1335)

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SKU:
CAB1335
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cardiovascular

Description

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Antibody Name:Anti-VWF Antibody
Antibody SKU:CAB1335
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthetic peptide corresponding to a sequence within amino acids 50-150 of human VWF (NP_000543.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:1000
Reactivity:Human, Mouse, Rat
Positive Samples:
Immunogen:A synthetic peptide corresponding to a sequence within amino acids 50-150 of human VWF (NP_000543.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MYSF AGYC SYLL AGGC QKRS FSII GDFQ NGKR VSLS VYLG EFFD IHLF VNGT VTQG DQRV SMPY ASKG LYLE TEAG YYKL SGEA YGFV ARID GSGN FQVL L
Gene ID:7450
Uniprot:P04275
Cellular Location:Secreted, extracellular matrix, extracellular space
Calculated MW:38kDa/309kDa
Observed MW:Refer to figures
Synonyms:VWF, F8VWF, VWD
Background:This gene encodes a glycoprotein involved in hemostasis. The encoded preproprotein is proteolytically processed following assembly into large multimeric complexes. These complexes function in the adhesion of platelets to sites of vascular injury and the transport of various proteins in the blood. Mutations in this gene result in von Willebrand disease, an inherited bleeding disorder. An unprocessed pseudogene has been found on chromosome 22.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Cell adhesion; Secreted, signal peptide; Extracellular matrix; Motility/polarity/chemotaxis; 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; liver development; blood coagulation; cell adhesion; 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

NCBI Summary: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]
UniProt Code:P04275
NCBI GenInfo Identifier:317373549
NCBI Gene ID:7450
NCBI Accession:P04275.4
UniProt Secondary Accession:P04275,Q8TCE8, Q99806,
UniProt Related Accession:P04275
Molecular Weight:Calculated MW: 38kDa/309kDaObserved MW: Refer to figures
NCBI Full Name:von Willebrand factor
NCBI Synonym Full Names:von Willebrand factor
NCBI Official Symbol:VWF  
NCBI Official Synonym Symbols:VWD; F8VWF  
NCBI Protein Information:von Willebrand factor; coagulation factor VIII VWF
UniProt Protein Name:von Willebrand factor
UniProt Synonym Protein Names:von Willebrand antigen II
Protein Family:von Willebrand factor
UniProt Gene Name:VWF  
UniProt Entry Name:VWF_HUMAN
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