Sheep Factor VIII:C Polyclonal Antibody | anti-F8 antibody

Sheep anti-canine Factor VIII, Peroxidase Conjugated IgG

Cat. Num. AAA512215

• Gene Names: F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E
• Purity: Peroxidase conjugated IgG.; Vial containing ml of IgG conjugated to horseradish peroxidase (HRP) through carbohydrate groups. Total protein is 0.2 mg.
• Synonyms: Factor VIII:C; Polyclonal Antibody; Sheep anti-canine Factor VIII; Peroxidase Conjugated IgG; canine; anti-F8 antibody

• Host: Sheep
• Clonality: Polyclonal
• Specificity: Prior to conjugation, this antibody was specific for canine FVIII as demonstrated by immunoelectrophoresis and ELISA.
• Purity/Purification: Peroxidase conjugated IgG.; Vial containing ml of IgG conjugated to horseradish peroxidase (HRP) through carbohydrate groups. Total protein is 0.2 mg.
• Form/Format: IgG-HRP conjugate as a clear, slightly red-brown liquid.
• Concentration: IgG-HRP concentration is mg/ml, determined by absorbance using an extinction coefficient (E1%280) of 14. (varies by lot)

• Application Notes: Suitable as a source of peroxidase-labeled antibodies to canine FVIII.
• Immunogen: Recombinant canine FVIII (cFVIII).
• Buffer: A buffered stabilizer solution containing 50% (v/v) glycerol.
• Preparation and Storage: Store between -10 and -20 degree C. Product will become viscous but will not freeze. Avoid storage in frost-free freezers. Keep vial tightly capped. Allow product to warm to room temperature and gently mix before use. Avoid exposure to sodium azide as this is an inhibitor of peroxidase activity.

Related Product Information for anti-F8 antibody

Factor VIII is a large glycoprotein (320 kDa) synthesized in the liver. The majority of Factor VIII is cleaved during expression, resulting in a mixture of partially cleaved forms ranging in size from 200-280 kDa. The FVIII is stabilized in circulation through non-covalent association with von Willebrand Factor. The concentration of FVIII in normal human plasma is typically 200 ng/mL. In canine plasma, the FVIII activity is 5-7 fold higher relative to human plasma. FVIII is a pro-cofactor that is activated through limited proteolysis by thrombin. In this process FVIIIa dissociates from vWF to combine with activated Factor IX, calcium and a phospholipid surface where it is an essential cofactor in the assembly of the Factor X activator complex. Hemophilia A is a congenital bleeding disorder resulting from an X-chromosome-linked deficiency of FVIII, occurring with a frequency of 1 in 4000 males. The defect can be caused by any one of hundreds of reported mutations but are most commonly due to inversions within intron 22 of the FVIII gene. Hemophilia A has also been reported in a variety of species including dog and mouse, with a clinical phenotype very similar to human. The genetic defect in one case of canine Hemophilia-A has been shown to also be due to a gene inversion similar to the human defect, possibly indicating a common instability of the FVIII gene in humans and dogs1-4.

References

1. Lollar P, Fay PJ, Fass DN; Factor VIII and Factor VIIIa. Methods in Enzymology, 222, pg 122, 1993.
2. Hoyer, LW, Wyshock EG, Colman RW, in Hemostasis and Thrombosis, 3rd Edition, eds. RW Colman, J Hirsh, VJ Marder and EW Salzman, pp. 109-133, J.B. Lippincott Co., Philadelphia, 1994.
3. Lozier JN, Dutra A. et al: The Chapel Hill hemophilia dog colony exhibits a factor VIII gene inversion. PNAS. 99, pg 12991-96, 2002.
4. Gallo-Penn AM, Shirley PS. et al: Systemic delivery of an adeno-viral viral vector encoding canine factor VIII results in short term phenotypic correction, inhibitor development and liver toxicity in hemophilic dogs. Blood. 97, pg 107-113, 2001.

NCBI and Uniprot Product Information

• NCBI GI #: 182803
• NCBI GeneID: 2157
• UniProt Accession #: P00451; Q14286; Q5HY69
• Molecular Weight: 267,009 Da
• NCBI Official Full Name: factor VIII
• NCBI Official Synonym Full Names: coagulation factor VIII, procoagulant component
• NCBI Official Symbol: F8
• NCBI Official Synonym Symbols: AHF; F8B; F8C; HEMA; FVIII; DXS1253E
• NCBI Protein Information: coagulation factor VIII; factor VIII F8B; antihemophilic factor; coagulation factor VIIIc
• UniProt Protein Name: Coagulation factor VIII
• Protein Family: Coagulation factor
• UniProt Gene Name: F8
• UniProt Synonym Gene Names: F8C; AHF
• UniProt Entry Name: FA8_HUMAN

NCBI Description

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 Description

Function: 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.

Subunit structure: Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation. Ref.14 Ref.15

Subcellular location: Secreted › extracellular space.

Domain: Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity.

Post-translational modification: Sulfation on Tyr-1699 is essential for binding vWF.

Involvement in 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. Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.35 Ref.36 Ref.37 Ref.38 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.45 Ref.46 Ref.47 Ref.48 Ref.49 Ref.50 Ref.51 Ref.52 Ref.53 Ref.54 Ref.55 Ref.56 Ref.57 Ref.58 Ref.59 Ref.60 Ref.61 Ref.62 Ref.63 Ref.64 Ref.65 Ref.66 Ref.67 Ref.68 Ref.69 Ref.70 Ref.71 Ref.72 Ref.73 Ref.74 Ref.75 Ref.76 Ref.77 Ref.78 Ref.79 Ref.80 Ref.81 Ref.82 Ref.83 Ref.84 Ref.86 Ref.87 Ref.88 Ref.89 Ref.90 Ref.91 Ref.92 Ref.93 Ref.94 Ref.95 Ref.96 Ref.97 Ref.98 Ref.99 Ref.100 Ref.101

Pharmaceutical use: Available under the names Kogenate (Bayer) and Recombinate (Baxter and American Home Products). Used to treat hemophilia A.

Sequence similarities: Belongs to the multicopper oxidase family.Contains 3 F5/8 type A domains.Contains 2 F5/8 type C domains.Contains 6 plastocyanin-like domains.

Mass spectrometry: Molecular mass is 1367.6 Da from positions 356 - 378. Determined by ESI. Nonsulfated. Ref.12Molecular mass is 1407.4 Da from positions 356 - 378. Determined by ESI. Sulfated. Ref.12Molecular mass is 2975.4 Da from positions 400 - 424. Determined by ESI. Nonsulfated. Ref.12Molecular mass is 3024 Da from positions 727 - 752. Determined by ESI. Nonsulfated. Ref.12Molecular mass is 3104 Da from positions 727 - 752. Determined by ESI. Monosulfated. Ref.12Molecular mass is 3183.5 Da from positions 727 - 752. Determined by ESI. Disulfated. Ref.12Molecular mass is 3262.5 Da from positions 727 - 752. Determined by ESI. Trisulfated. Ref.12Molecular mass is 2460.9 Da from positions 1672 - 1692. Determined by ESI. Nonsulfated. Ref.12Molecular mass is 2540.7 Da from positions 1672 - 1692. Determined by ESI. Sulfated. Ref.12Molecular mass is 2000.7 Da from positions 1691 - 1708. Determined by ESI. Nonsulfated. Ref.12Molecular mass is 2080.7 Da from positions 1691 - 1708. Determined by ESI. Sulfated. Ref.12