Mouse Factor VIII Related Antigen (FVIII-Ag) ELISA Kit (RMEK-0153724)
Cat. No.: RMEK-0153724
Category: ELISA Kits
INQUIRY
1 x 96 tests
This ELISA kit is a 1. 5 hour solid-phase ELISA designed for the quantitative determination of the targets. This ELISA kit for research use only, not for therapeutic or diagnostic applications!
Product Features
| Species Reactivity | Mouse |
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| Assay Time | 1.5 h |
Target Information
| Target Symbol | F8 |
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| UniProt ID | P00451 |
| Function | Factor VIII, along with calcium and phospholipid, acts as a cofactor for F9/factor IXa when it converts F10/factor X to the activated form, factor Xa. |
| Cellular Localization | Secreted, extracellular space. |
| Domain | Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity. |
| Post-transcriptional Modifications | Sulfation on Tyr-1699 is essential for binding vWF. Proteolytically cleaved by cathepsin CTSG to produce a partially activated form. |
| Involvement in Disease | 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. The disease is caused by variants affecting the gene represented in this entry. 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. Thrombophilia 13, X-linked, due to factor VIII defect (THPH13): An X-linked dominant, hemostatic disorder associated with markedly elevated F8 levels, and characterized by severe thrombophilia. The disease is caused by variants affecting the gene represented in this entry. |
Storage & Shipping
| Storage | Store at 2-8°C |
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| Shipping | Gel Packs |
| Stability | The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition. |
For research use only. Not for clinical use.
