antibody

Sheep anti-human Factor V (F.V), Whole IgG from antiserum

MBS512038

10 mg

280 USD

polyclonal

sheep

nonconjugated

Antibody

Sheep anti-human Factor V (F.V), Whole IgG from antiserum

Factor V

Factor V, human

factor V precusor; Coagulation factor V; coagulation factor V; factor V Leiden; OTTHUMP00000032547; OTTHUMP00000032548; proaccelerin, labile factor; activated protein c cofactor; coagulation factor V jinjiang A2 domain; coagulation factor V (proaccelerin, labile factor); Activated protein C cofactor; Proaccelerin, labile factor

F5; F5; FVL; PCCF

FA5_HUMAN

Polyclonal

Sheep

This antibody is specific for factor V as demonstrated by immunoelectrophoresis and ELISA.

Purified IgG. Vial containing ml of whole IgG representing approximately 1 ml of antiserum. Total protein is 10 mg.

Whole IgG, clear liquid.

IgG concentration is mg/ml, determined by absorbance using an extinction coefficient (E1%280) of 13.4.

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.

Suitable as a source of antibodies to human factor V.

Immunogen: Human factor V purified from plasma.

Buffer: 10 mM HEPES, pH 7.4, 150 mM NaCl, 50% (v/v) glycerol. Neutralizing Activity: XXXX Bethesda Units/ml IgG against normal plasma. One Bethesda unit/ml is defined as the amount of inhibitor that resulted in 50% residual F.V activity after 2 hours at 37 degree C (Kasper CK et al, Thromb Diath Haemorrh 34:869, 1975).

Factor V (formerly referred to as accelerator globulin and labile factor) is a large glycoprotein (320 kDa) that is produced in the liver. The gene that encodes factor V (F.V) is located on chromosome 1. A congenital deficiency of F.V is a hemorrhagic disorder inherited as an autosomal recessive disease. The concentration of F.V in plasma is typically 10 ug/ml. F.V is a pro-cofactor that is activated through limited proteolysis by thrombin, or by activated factor X in the presence of phospholipid surface. Other physiologic activators of F.V include plasmin, neutrophil elastase and platelet calpain. The activated cofactor (F.Va) is an essential component of the prothrombin activator complex, which consists of F.Va, activated factor X, calcium and anionic phospholipid surface. The intact prothrombinase complex activates prothrombin to thrombin at a rate 300,000-fold greater than activated factor X alone. In a positive feedback loop, the thrombin generated accelerates its own generation by activating more F.V to F.Va. Thrombin also acts to down-regulate F.Va indirectly by activating Protein C, which inactivates F.Va cofactor activity1-3.

1. Kane WH, Davie EW; Blood Coagulation Factors V and VIII: Structural and functional similarities and their relationship to hemorrhagic and thrombotic disorders. Blood 71:539, 1988. 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. Nesheim ME, Katzmann JA, Tracy PB, Mann KG; in Methods in Enzymology 80:249, 1980.

182798

P12259

251,703 Da

Blood Clotting Cascade Pathway 198840!!Common Pathway 106060!!Complement And Coagulation Cascades Pathway 198880!!Complement And Coagulation Cascades Pathway 83073!!Complement And Coagulation Cascades Pathway 484!!Formation Of Fibrin Clot (Clotting Cascade) Pathway 106057!!Formation Of Platelet Plug Pathway 106029!!Hemostasis Pathway 106028!!Platelet Activation Pathway 106034!!Platelet Degranulation Pathway 106050

This gene encodes an essential cofactor of the blood coagulation cascade. This factor circulates in plasma, and is converted to the active form by the release of the activation peptide by thrombin during coagulation. This generates a heavy chain and a light chain which are held together by calcium ions. The activated protein is a cofactor that participates with activated coagulation factor X to activate prothrombin to thrombin. Defects in this gene result in either an autosomal recessive hemorrhagic diathesis or an autosomal dominant form of thrombophilia, which is known as activated protein C resistance. [provided by RefSeq]

Function: Central regulator of hemostasis. It serves as a critical cofactor for the prothrombinase activity of factor Xa that results in the activation of prothrombin to thrombin. Subunit structure: Factor Va, the activated form of factor V, is composed of a heavy chain and a light chain, non-covalently bound. The interaction between the two chains is calcium-dependent. Subcellular location: Secreted. Tissue specificity: Plasma. Ref.18. Domain: Domain B contains 35 x 9 AA tandem repeats, and 2 x 17 AA repeats. Post-translational modification: Thrombin activates factor V proteolytically to the active cofactor, factor Va (formation of a heavy chain at the N-terminus and a light chain at the C-terminus).Sulfation is required for efficient thrombin cleavage and activation and for full procoagulant activity.Activated protein C inactivates factor V and factor Va by proteolytic degradation.Phosphorylation sites are present in the extracelllular medium. Involvement in disease: Defects in F5 are the cause of factor V deficiency (FA5D) [. MIM:227400]; also known as Owren parahemophilia. It is an hemorrhagic diastesis. Ref.30 Ref.33Defects in F5 are the cause of thrombophilia due to activated protein C resistance (THR-APCR) [. MIM:188055]. THR-APCR is a hemostatic disorder due to defective degradation of factor Va by activated protein C. It is characterized by a poor anticoagulant response to activated protein C resulting in tendency to thrombosis. Ref.26 Ref.31 Ref.32 Ref.34 Ref.36Defects in F5 are a cause of susceptibility to Budd-Chiari syndrome (BCS) [. MIM:600880]. It is a syndrome caused by obstruction of hepatic venous outflow involving either the hepatic veins or the terminal segment of the inferior vena cava. Obstructions are generally caused by thrombosis and lead to hepatic congestion and ischemic necrosis. Clinical manifestations observed in the majority of patients include hepatomegaly, right upper quadrant pain and abdominal ascites. Budd-Chiari syndrome is associated with a combination of disease states including primary myeloproliferative syndromes and thrombophilia due to factor V Leiden, protein C deficiency and antithrombin III deficiency. Budd-Chiari syndrome is a rare but typical complication in patients with polycythemia vera.Defects in F5 may be a cause of susceptibility to ischemic stroke (ISCHSTR) [. MIM:601367]; also known as cerebrovascular accident or cerebral infarction. A stroke is an acute neurologic event leading to death of neural tissue of the brain and resulting in loss of motor, sensory and/or cognitive function. Ischemic strokes, resulting from vascular occlusion, is considered to be a highly complex disease consisting of a group of heterogeneous disorders with multiple genetic and environmental risk factors. Ref.35. 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. Sequence caution: The sequence ABD23003.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI23065.1 differs from that shown. Reason: Erroneous gene model prediction.

10 mg

280 USD