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You are here:Home » Antibodies » Abs to Coagulation Factors » Anti -Factor VIII

Anti -Factor VIII

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Specifications

Clone Host Grade Applications
Monoclonal Mouse Purified B
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. It is an extracellular factor. Defects in F8 are the cause of hemophilia A (HEMA). HEMA is a common recessive X linked coagulation disorder. The frequency of hemophilia A is 1-2 in 10,000 male births in all ethnic groups. About 50% of patients have severe hemophilia A with F8C activity less than 1% of normal; they have frequent spontaneous bleeding into joints, muscles and internal organs. Moderately severe hemophilia A occurs in about 10% of patients; F8C activity is 2-5% of normal, and there is bleeding after minor trauma. Mild hemophilia A, which occurs in 30-40% of patients, is associated with F8C activity of 5-30% and bleeding occurs only after significant trauma or surgery. Of particular interest for the understanding of the function of F8C is the category of CRM (cross-reacting material) positive patients (~5%) that have considerable amount of F8C in their plasma (at least 30% of normal), but the protein is nonfunctional; i.e., the F8C 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.
Catalog #F0016-07C
ApplicationsSuitable for use in Western Blot. Other applications not tested.
Recommended DilutionWestern Blot: 1:25-1:200
Optimal dilutions to be determined by the researcher.
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypeMonoclonal
IsotypeIgG1
Clone No8k141
HostMouse
SourceHuman
ConcentrationAs reported
FormSupplied as a liquid in PBS, pH 7.2, 50% glycerol. No preservative added.
PurityPurified
ImmunogenHuman Factor VIII.
SpecificityRecognizes the 360kD band of human factor VIII as well as the 80kD doublet bands. Species Crossreactivity: porcine. Does not cross-react with von Willebrand factor.
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.


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