Factor VIII antibody | MyBioSource MBS531036 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Factor VIII antibody

catalog :

MBS531036

quantity :

1 mg

price :

980 USD

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

10101

reactivity :

human

application :

western blot, ELISA, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS531036

products type :

Antibody

products full name :

Factor VIII antibody

products short name :

Factor VIII

products name syn :

Monoclonal Factor VIII; Anti-Factor VIII; Complement Factor VIII

other names :

factor VIII; Coagulation factor VIII; coagulation factor VIII; coagulation factor VIII, procoagulant component; Antihemophilic factor; AHF

other gene names :

F8; F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E; F8C; AHF

uniprot entry name :

FA8_HUMAN

clonality :

Monoclonal

isotype :

IgG1

clone :

10101

host :

Mouse

reactivity :

Human factor VIII

sequence length :

2351

form :

Purified IgG protein supplied in PBS.

concentration :

0.7mg/ml

storage stability :

Aliquot and store at -70 degree C or lower. Avoid repeated freeze/thaw cycles

tested application :

ELISA (EIA), Western Blot (WB)

app notes :

ELISA: 1:16,000

other info1 :

Biological Significance: Factor VIII (FVIII) is an essential blood clotting factor also known as anti-hemophilic factor (AHF). In humans, Factor VIII is encoded by the F8 gene. Defects in this gene results in hemophilia A, a well known recessive X-linked coagulation disorder. Factor VIII participates in blood coagulation; it is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids forms a complex that converts factor X to the activated form Xa.

other info2 :

Immunogen: Factor Vlll antibody was raised in mouse using purified human factor VIII as the immunogen.

products categories :

Immunology

products description :

Mouse monoclonal Factor VIII antibody

ncbi gi num :

182803

ncbi acc num :

AAA52484.1

ncbi mol weight :

24,641 Da

ncbi pathways :

Blood Clotting Cascade Pathway (198840); Common Pathway Of Fibrin Clot Formation (106060); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); Hemostasis Pathway (106028); Intrinsic Pathway Of Fibrin Clot Formation (106059); Platelet Activation, Signaling And Aggregation Pathway (106034); Platelet Degranulation Pathway (106050)

ncbi summary :

This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder. [provided by RefSeq, Jul 2008]

uniprot summary :

F8: Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa. Defects in F8 are the cause of hemophilia A (HEMA). A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non- functional; i.e. the F8 activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein. Belongs to the multicopper oxidase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Secreted, signal peptide; Secreted. Chromosomal Location of Human Ortholog: Xq28. Cellular Component: extracellular space; extracellular region; plasma membrane. Molecular Function: protein binding; copper ion binding; serine-type endopeptidase activity; oxidoreductase activity. Biological Process: platelet activation; platelet degranulation; acute-phase response; blood coagulation; proteolysis; blood coagulation, intrinsic pathway. Disease: Hemophilia A; Factor Viii Deficiency

size1 :

1 mg

price1 :

980 USD

more info or order :

MyBioSource product webpage