antibody

Sheep anti-human Factor VII (FVII), FITC-Conjugated Affinity-Purified IgG

MBS512049

0.1 mg

380 USD

polyclonal

sheep

FITC

Antibody

Sheep anti-human Factor VII (FVII), FITC-Conjugated Affinity-Purified IgG

[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

Prior to conjugation, this antibody was specific for Factor VII as demonstrated by immunoelectrophoresis and ELISA.

Affinity purified, FITC conjugated IgG. Vial containing 0.1 mL of affinity-purified IgG conjugated to fluorescein isothiocyanate (FITC). Total protein is 0.1 mg.

APIgG-FITC conjugate as a clear yellow liquid.

APIgG-FITC concentration is 1.0 mg/mL, determined by absorbance using an extinction coefficient (E 1% 280 ) of 14.

Store at 2 degree C to 8 degree C and protect from light.

This reagent is suitable as a source of fluorescein labeled antibodies to Factor VII.

Testing Data

Immunogen: Human FVII purified from plasma. Specifications: Vial containing 0.1 mL of affinity-purified IgG conjugated to fluorescein isothiocyanate (FITC). Total protein is 0.1 mg.

Buffer: Phosphate-buffered saline containing 1 mg/mL bovine albumin and 0.1% sodium azide (w/v), pH 7.4. Incorporation of FITC: 3.21 moles fluorscein per mole IgG as determined spectrophotometrically.

Factor VII (FVII, also known as Stable Factor and Proconvertin) is a vitamin K-dependent glycoprotein produced in the liver. Plasma concentration of FVII is normally ~0.5 ug/mL (10 nM) in plasma. A deficiency of FVII 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 FVII function. In its zymogen form FVII 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 FVII to bind divalent metal ions and participate in calcium-dependent binding interactions. FVII and activated FVII (FVIIa) bind to tissue factor exposed at the site of vascular injury. FIXa, FXa or FVIIa rapidly activate tissue factor-bound FVII to FVIIa in the presence of calcium and phospholipid. Thrombin and FXIIa are able to activate FVII in the fluid phase in the absence of cofactors. The activation of the single chain zymogen FVII occurs by proteolysis after residue Arg152, resulting in a two-chain active serine protease consisting of a 30 kDa heavy chain and an 18 kDa light chain. In complex with tissue factor, phospholipid and calcium, FVIIa is able to activate FX and FIX. Free FVIIa in plasma is remarkably stable, but the activity of FVIIa/TF complex is regulated by Tissue Factor Pathway Inhibitor (TFPI) in the presence of FXa, 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. 4. Broze, GJ; Binding of Human Factor VII and VIIa to Monocytes. J. Clin. Invest, pp 526-535, 1982.

180334

P08709

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.

0.1 mg

380 USD