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Human F8 Recombinant Protein (RPPB3641)

SKU:
RPPB3641
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
P00451
€1,549
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Description

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Product Name:Human F8 Recombinant Protein
Product Code:RPPB3641
Size:400IU
Species:Human
Target:F8
Synonyms:Coagulation factor VIII, Procoagulant component, Antihemophilic factor, AHF, F8, F8C, F8B, HEMA, FVIII, DXS1253E, F8 protein.
Source:Human Plasma
Physical Appearance:Sterile Filtered White lyophilized (freeze-dried) powder.
Formulation:The lyophilized protein 200IU/ml was lyophilized from a sterile solution containing 1.5% Glycine, 160mM Calcium chloride and 25mM NaCitrate and 25mM NaCl.
Solubility:It is recommended to reconstitute the lyophilized Factor-VIII in sterile 18M?-cm H2O at a concentration of 200IU/ml, which can then be further diluted to other aqueous solutions.Make sure that the vial has reached room temperature prior to its reconstitution, otherwise it might precipitate.
Stability:Lyophilized Factor-VIII although stable at room temperature for 1 week, should be stored desiccated between 2-8°C. Upon reconstitution Factor-VIII should be stored at 4°C.
Biological Activity:The potency was found to be 10 Units/mg.

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.

Human Factor VIII produced from Human Plasma contains 2332 amino acids and having a molecular mass of 330kDa. Factor-VIII is effective in the correction and prevention of severe bleeding episodes attributed to Factor VIII deficiency.The Factor-VIII is purified by proprietary chromatographic techniques.

UniProt Protein Function: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.
UniProt Protein Details:

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; proteolysis; blood coagulation; blood coagulation, intrinsic pathway

Disease: Hemophilia A; Factor Viii Deficiency

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 Code:P00451
NCBI GenInfo Identifier:119767
NCBI Gene ID:2157
NCBI Accession:P00451.1
UniProt Secondary Accession:P00451,Q14286, Q5HY69,
UniProt Related Accession:P00451
Molecular Weight:24,641 Da
NCBI Full Name:Coagulation factor VIII
NCBI Synonym Full Names:coagulation factor VIII, procoagulant component
NCBI Official Symbol:F8��
NCBI Official Synonym Symbols:AHF; F8B; F8C; HEMA; FVIII; DXS1253E��
NCBI Protein Information:coagulation factor VIII; factor VIII F8B; antihemophilic factor; coagulation factor VIIIc
UniProt Protein Name:Coagulation factor VIII
UniProt Synonym Protein Names: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
Protein Family:Factor VIII intron 22 protein
UniProt Gene Name:F8��
UniProt Entry Name:FA8_HUMAN

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