protein

Recombinant Human Coagulation Factor VIII

MBS142874

250 IU

1375 USD

Recombinant Protein

Recombinant Human Coagulation Factor VIII

Coagulation Factor VIII

F8 Human; Coagulation Factor-VIII Human Recombinant; Coagulation factor VIII; Procoagulant component; Antihemophilic factor; AHF; F8; F8C; F8B; HEMA; FVIII; DXS1253E; F8 protein

coagulation factor VIII isoform a; Coagulation factor VIII; coagulation factor VIII; antihemophilic factor; coagulation factor VIIIc; factor VIII F8B; coagulation factor VIII, procoagulant component; Antihemophilic factor; AHF; Procoagulant componentCleaved into the following 4 chains:Factor VIIIa heavy chain, 200 kDa isoform; Factor VIIIa heavy chain, 92 kDa isoform; Factor VIII B chain; Factor VIIIa light chain

F8

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

FA8_HUMAN

Chinese Hamster Ovarian Cells (CHO)

2351

Greater than 97.0% as determined by SDS-PAGE.

The protein was lyophilized from a solution containing 50mM histidine, 0.3M NaCl, 5mM CaCl2, 0.02% Tween 80, 20mM sucrose, pH- 6.8. Sterile Filtered White lyophilized (freeze-dried) powder.

Lyophilized Factor-VIII although stable at room temperature for 3 weeks, should be stored desiccated below -18 degree C. Upon reconstitution Factor-VIII should be stored at 4 degree C between 2-7 days and for future use below -18 degree C.Please prevent freeze-thaw cycles.

Solubility: It is recommended to reconstitute 250IU lyophilized Factor-VIII in 5ml sterile 18M-cm H2O, which can then be further diluted to other aqueous solutions. Biological Activity: The specific activity was found to be 7058IU/mg.

RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins; Coagulation Factors

Description: Antihemophilic Facor Human Recombinant produced in CHO is a glycosylated polypeptide chain having a total amino acids of 1438 (170kd) and consisting of two dimer chains 80 kD and 90 kD.The Factor-VIII lacks central region of B-domain region and is purified by proprietary chromatographic techniques. Introduction: Coagulation factor VIII 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.

4503647

NP_000123.1

NM_000132.3

P00451

24,641 Da

Blood Clotting Cascade Pathway (198840); Common Pathway (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 (106059); Platelet Activation, Signaling And Aggregation Pathway (106034); Platelet Degranulation Pathway (106050)

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]

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; Secreted, signal peptide. 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

250 IU

1375 USD

500 IU

2460

1000 IU

4140