antibody

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

MBS512046

10 mg

280 USD

polyclonal

sheep

nonconjugated

Antibody

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

Factor VII

Factor VII, human

factor VII; Coagulation factor VII; coagulation factor VII; SPCA; eptacog alfa; proconvertin; OTTHUMP00000018733; OTTHUMP00000018734; FVII coagulation protein; serum prothrombin conversion accelerator; coagulation factor VII (serum prothrombin conversion accelerator); Proconvertin; Serum prothrombin conversion accelerator

F7; F7

FA7_HUMAN

Polyclonal

Sheep

This antibody is specific for factor VII 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 VII.

Immunogen: Human F.VII purified from plasma.

Buffer: 10 mM HEPES, pH 7.4, 150 mM NaCl, 50% (v/v) glycerol. Neutralizing Activity: Not determined.

Factor VII (F.VII, also known as Stable Factor and Proconvertin) is a vitamin K-dependent glycoprotein produced in the liver. Plasma concentration of F.VII is normally ~0.5 ug/ml (10 nM) in plasma. A deficiency of F.VII is associated with bleeding in a clinical pattern similar to haemophilia, but is inherited as an autosomal recessive trait. The deficiency can be characterized by a quantitative (low activity and low antigen) or a qualitative (low activity and normal antigen) defect in F.VII function. In its zymogen form F.VII is a single chain molecule of ~50 kDa. It contains two EGF-like domains and an amino-terminal domain containing 10 gamma-carboxyglutamic acid (Gla) residues. These Gla residues allow F.VII to bind divalent metal ions and participate in calcium-dependent binding interactions. F.VII and activated F.VII (F.VIIa) bind to tissue factor exposed at the site of vascular injury. F.IXa, F.Xa or F.VIIa rapidly activate tissue factorbound F.VII to F.VIIa in the presence of calcium and phospholipid. Thrombin and F.XIIa are able to activate F.VII in the fluid phase in the absence of cofactors. The activation of the single chain zymogen F.VII occurs by proteolysis after residue Arg152, resulting in a twochain active serine protease consisting of a 30 kDa heavy chain and an 18 kDa light chain. In complex with tissue factor, phospholipid and calcium, F.VIIa is able to activate F.X and F.IX. Free F.VIIa in plasma is remarkably stable, but the activity of F.VIIa/TF complex is regulated by Tissue Factor Pathway Inhibitor (TFPI) in the presence of F.Xa, and also by Antithrombin (ATIII) in the presence of heparin1-3.

1. Rao LVM, Bajaj SP, Rapaport SI; Activation of Human Factor VII During Clotting in Vitro; Blood 65, pp 218-226, 1985. 2. Lawson, JH, Butenas S, Ribarik N, Mann KG; Complex-dependent Inhibition of Factor VIIa by Antithrombin III and Heparin; JBC 268 pp 767-770, 1993. 3. Nemerson Y, in Hemostasis and Thrombosis, 3rd Edition, eds. RW Colman, J Hirsh, VJ Marder and EW Salzman, pp. 81-93, J.B. Lippincott Co., Philadelphia PA, USA, 1994.

180334

P08709

51,594 Da

Blood Clotting Cascade Pathway (198840); Circadian Clock Pathway (187173); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Extrinsic Pathway (106058); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); Gamma-carboxylation Of Protein Precursors Pathway (106233); Gamma-carboxylation, Transport, And Amino-terminal Cleavage Of Proteins Pathway (106232); Hemostasis Pathway (106028)

This gene encodes coagulation factor VII which is a vitamin K-dependent factor essential for hemostasis. This factor circulates in the blood in a zymogen form, and is converted to an active form by either factor IXa, factor Xa, factor XIIa, or thrombin by minor proteolysis. Upon activation of the factor VII, a heavy chain containing a catalytic domain and a light chain containing 2 EGF-like domains are generated, and two chains are held together by a disulfide bond. In the presence of factor III and calcium ions, the activated factor then further activates the coagulation cascade by converting factor IX to factor IXa and/or factor X to factor Xa. Alternative splicing of this gene results in 2 transcripts. Defects in this gene can cause coagulopathy. [provided by RefSeq]

Function: Initiates the extrinsic pathway of blood coagulation. Serine protease that circulates in the blood in a zymogen form. Factor VII is converted to factor VIIa by factor Xa, factor XIIa, factor IXa, or thrombin by minor proteolysis. In the presence of tissue factor and calcium ions, factor VIIa then converts factor X to factor Xa by limited proteolysis. Factor VIIa will also convert factor IX to factor IXa in the presence of tissue factor and calcium. Catalytic activity: Selective cleavage of Arg- -Ile bond in factor X to form factor Xa. Subunit structure: Heterodimer of a light chain and a heavy chain linked by a disulfide bond. Subcellular location: Secreted. Tissue specificity: Plasma. Post-translational modification: The vitamin K-dependent, enzymatic carboxylation of some glutamate residues allows the modified protein to bind calcium.The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. Involvement in disease: Defects in F7 are the cause of factor VII deficiency (FA7D) [. MIM:227500]. A hemorrhagic disease with variable presentation. The clinical picture can be very severe, with the early occurrence of intracerebral hemorrhages or repeated hemarthroses, or, in contrast, moderate with cutaneous-mucosal hemorrhages (epistaxis, menorrhagia) or hemorrhages provoked by a surgical intervention. Finally, numerous subjects are completely asymptomatic despite very low factor VII levels. Ref.11 Ref.18 Ref.19 Ref.20 Ref.22 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.35 Ref.36 Ref.37 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.45. Pharmaceutical use: Available under the names Niastase or Novoseven (Novo Nordisk). Used for the treatment of bleeding episodes in hemophilia A or B patients with antibodies to coagulation factors VIII or IX. Sequence similarities: Belongs to the peptidase S1 family.Contains 2 EGF-like domains.Contains 1 Gla (gamma-carboxy-glutamate) domain.Contains 1 peptidase S1 domain.

10 mg

280 USD