catalog number :
MBS2010586
products type :
Recombinant Protein
products full name :
Recombinant Coagulation Factor VIII (F8)
products short name :
Coagulation Factor VIII (F8)
other names :
coagulation factor VIII isoform a; Coagulation factor VIII; coagulation factor VIII; factor VIII F8B; antihemophilic factor; coagulation factor VIIIc; coagulation factor VIII, procoagulant component; Antihemophilic factor; AHF; Procoagulant component
other gene names :
F8; F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E; F8C; AHF
uniprot entry name :
FA8_HUMAN
sequence :
The target protein is fused with N-terminal His-Tag, its sequence is listed below. MGHHHHHHSGSEF-GRQV TVQEFALFFT IFDETKSWYF TENMERNCRA PCNIQMEDPT FKENYRFHAI NGYIMDTLPG LVMAQDQRIR WYLLSMGSNE NIHSIHFSGH VFTVRKKEEYKMALYNLYPG VFETVEMLPS KAGIWRVECL IGEHLHAGMS TLFLVYSNKC QTPL
form :
Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.
storage stability :
Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months. Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.
tested application :
SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
app notes :
WB: 1:500 - 1:1000
other info1 :
Organism: Homo sapiens (Human). Expression System: Prokaryotic expression. Residues: Gly1887~Leu2044 (Accession # P00451) with N-terminal His-Tag. Subcellular Location: Secreted, extracellular space. Immunogen: Recombinant protein of human RALBP1
other info2 :
Endotoxin Level: <1.0EU per 1ug (determined by the LAL method). Predicted Isoelectric Point: 6.4. Reconstitution: Reconstitute in sterile PBS, pH7.2-pH7.4.
products description :
About the Marker: Effective Size Range: 10kDa to 70kDa. Protein bands: 10kDa, 14kDa, 18kDa, 22kDa, 26kDa, 33kDa, 44kDa and70kDa. Double intensity bands: The 26kDa, 18kDa, 10kDa bands are at doubleintensity to make location and size approximation of proteins of interestquick and easy. Ready-to-use: No need to heat, dilute or add reducing agents before use.
ncbi acc num :
NP_000123.1
ncbi gb acc num :
NM_000132.3
ncbi pathways :
Blood Clotting Cascade Pathway (198840); 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); Response To Elevated Platelet Cytosolic Ca2+ Pathway (106048)
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; Secreted, signal peptide. Chromosomal Location of Human Ortholog: Xq28. Cellular Component: extracellular space; plasma membrane; extracellular region. 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