catalog number :
MBS724256
products type :
ELISA Kit
products full name :
Rabbit Coagulation factor VIII related antigen ELISA Kit
products short name :
Coagulation factor VIII related antigen
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 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
products gene name :
F8-Ag
other gene names :
F8; F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E; F8C; AHF
uniprot entry name :
FA8_HUMAN
storage stability :
Store all reagents at 2-8 degree C.
other info1 :
Samples: Cell culture fluid & body fluid & tissue homogenate Serum or blood plasma
products description :
For Samples: Cell culture fluid & body fluid & tissue homogenate serum or blood plasma. Intended Uses: This F8R-Ag ELISA kit is intended for laboratory research use only and not for use in diagnostic or therapeutic procedures. The stop solution changes the color from blue to yellow and the intensity of the color is measured at 450 nm using a spectrophotometer. In order to measure the concentration of F8R-Ag in the sample, this F8R-Ag ELISA Kit includes a set of calibration standards. The calibration standards are assayed at the same time as the samples and allow the operator to produce a standard curve of Optical Density versus F8R-Ag concentration. The concentration of in the samples is then determined by comparing the O.D. of the samples to the standard curve. Principle of the Assay: The coated well immunoenzymatic assay for the quantitative measurement of F8R-Ag utilizes a polyclonal anti-F8R-Ag antibody and an F8R-Ag-HRP conjugate. The assay sample and buffer are incubated together with F8R-Ag-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the F8R-Ag concentration since F8R-Ag from samples and F8R-Ag-HRP conjugate compete for the anti-F8R-Ag antibody binding site. Since the number of sites is limited, as more sites are occupied by F8R-Ag from the sample, fewer sites are left to bind F8R-Ag-HRP conjugate.Standards of known F8R-Ag concentrations are run concurrently with the samples being assayed and a standard curve is plotted relating the intensity of the color (O.D.) to the concentration of F8R-Ag. The F8R-Ag concentration in each sample is interpolated from this standard curve.
ncbi acc num :
NP_000123.1
ncbi gb acc num :
NM_000132.3
ncbi mol weight :
24,641 Da
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; blood coagulation; proteolysis; blood coagulation, intrinsic pathway. Disease: Hemophilia A; Factor Viii Deficiency