Application
Western blot analysis of Factor VIII heavy chain expression in human kidney tissue extract.Application
Western blot analysis of Factor VIII heavy chain expression in human platelet whole cell lysate.| Product Name | Factor VIII heavy chain Antibody (H-140) |
|---|---|
| Antibody Type | Primary Antibodies |
| Modification Notes | Factor VIII is a glycoprotein cofactor that serves as a critical component in the blood coagulation pathway. Insufficient expression levels or expression of nonfunctional Factor VIII results in hemophilia A, a common severe hereditary bleeding disorder. In the liver, the main site of factor VIII synthesis, the mature polypetide chain of 2332 amino acids is secreted into the lumen of the endoplasmic reticulum, where it interacts with various chaperone proteins, including Calreticulin, Calnexin and IgG-binding protein. From the lumen, a portion of Factor VIII translocates to the Golgi and undergoes activation via proteolysis of both the heavy and light chain portions of the protein into three fragments. Finally, proteolysis of activated Factor VIII by Factor Xa, Protein C or Thrombin results in inactivation of Factor VIII. Survival of Factor VIII in the bloodstream requires binding to von Willebrand factor (VWF) at both the amino and carboxy termini of the light chain. Point mutations occuring in those binding domains as well as at other active sites of Factor VIII likely underly 90-95% of disease cases. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of Factor VIII precursor and 200 kDa and 92 kDa heavy chain isoforms of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine and bovine | |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 261-400 mapping within an internal region of Factor VIII of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 261-400 mapping within an internal region of Factor VIII of human origin |
| Entrez Gene | 2157 |
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Application
Western blot analysis of Factor VIII heavy chain expression in human kidney tissue extract.
Application
Western blot analysis of Factor VIII heavy chain expression in human platelet whole cell lysate.| Application Notes | recommended for detection of Factor VIII precursor and 200 kDa and 92 kDa heavy chain isoforms of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine and bovine |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
