factor VIII Rabbit pAb
factor VIII Rabbit pAb
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- 背景知识
Application
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | P00451 |
| Reactivity | Human |
| Host | Rabbit |
| Clonality | Polyclonal |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human factor VIII |
| Epitope Specificity | 1451-1550/2351 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Secreted, extracellular space. |
| DISEASE | Hemophilia A (HEMA) [MIM:306700]: 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. Note=The disease is caused by mutations 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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]. |
| Other Names | Coagulation factor VIII, Antihemophilic factor, AHF, Procoagulant component, Factor VIIIa heavy chain, 200 kDa isoform, Factor VIIIa heavy chain, 92 kDa isoform, Factor VIII B chain, Factor VIIIa light chain, F8, F8C |
|---|---|
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
REFERENCES
Truett M.A.,et al.DNA 4:333-349(1985).
Wood W.I.,et al.Nature 312:330-337(1984).
Levinson B.,et al.Genomics 14:585-589(1992).
Toole J.J.,et al.Nature 312:342-347(1984).
Gitschier J.,et al.Hum. Mol. Genet. 1:199-200(1992).
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