antibody

Goat anti-human Factor IX (FIX), Biotinylated Affinity-Purified IgG

MBS512034

0.1 mg

340 USD

polyclonal

goat

biotin

Antibody

Goat anti-human Factor IX (FIX), Biotinylated Affinity-Purified IgG

[Factor IX]

[Factor IX, human]

[factor IX; Coagulation factor IX; coagulation factor IX; FIX F9; factor 9; Christmas factor; OTTHUMP00000024154; plasma thromboplastic component; plasma thromboplastin component; coagulation factor IX; Christmas factor; Plasma thromboplastin component]

[F9; F9; FIX; P19; PTC; HEMB; MGC129641; MGC129642]

FA9_HUMAN

Polyclonal

Goat

Human

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

Affinity purified, Biotinylated IgG. Vial containing ml of affinity-purified IgG conjugated to biotin. Total protein is 0.1 mg.

APIgG-biotin conjugate as a clear, colourless liquid.

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

Store at 2-8°C

Suitable as a source of biotinylated antibodies to FIX.

Immunogen: Human Factor IX purified from plasma. Description: Vial containing 1 ml of affinity-purified IgG conjugated to biotin. Total protein is 0.1 mg.

Buffer: Phosphate-buffered saline containing 1 mg/ml bovine albumin and 0.1% sodium azide (w/v). Incorporation of Biotin: 5.7 moles biotin per mole IgG as determined by HABA assay

Factor IX (FIX, Christmas Factor) is a vitamin K-dependent glycoprotein produced in the liver. Plasma concentration of FIX is normally around 5 ug/ml (87 nM) in plasma. The biological importance of FIX is demonstrated in Haemophilia B (Christmas disease), an X-linked congenital bleeding disease resulting from a quantitative (low activity and low antigen) or qualitative (low activity and normal antigen) defect in FIX function. In its proenzyme or zymogen form FIX is a single chain molecule of 55,000 daltons. It contains two EGF-like domains and an amino-terminal domain containing 12 gamma-carboxy-glutamic acid (Gla) residues. These Gla residues allow FIX to bind divalent metal ions and participate in calcium-dependent binding interactions. The activation of FIX occurs by limited proteolysis in the presence of calcium by activated factor XI (FXIa) and/or by a complex of VIIa/tissue factor/phospholipid and activated Factor X between residues Arg146-Ala147 and between Arg180-Val181. The terminal activated product in either case is FIXabeta, a two-chain enzyme consisting of a heavy chain (28,000 daltons), a light chain (18,000 daltons) and an activation peptide product of 11,000 daltons. FIX can also be cleaved into inactive products by thrombin and by elastase. The activity of FIXabeta in plasma is inhibited by antithrombin and this inhibition is accelerated 1000-fold in the presence of optimal concentrations of heparin 1-3.

1. Lawson, JH, Mann KG; Cooperative Activation of Human FIX by the Human Extrinsic Pathway of Coagulation; JBC 266 pp11317-11327, 10991. 2. Enfield DL, Thompson AR; Cleavage and Activation of Factor IX by Serine Proteases; Blood 64, pp 821-831, 1984. 3. Limentani SA, Furie BC, Furie B, in Hemostasis and Thrombosis, 3rd Edition, eds. RW Colman, J Hirsh, VJ Marder and EW Salzman, pp. 94-108, J.B. Lippincott Co., Philadelphia PA, USA, 1994.

182609

P00740

51,778 Da

Blood Clotting Cascade Pathway (198840); 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); Intrinsic Pathway (106059)

This gene encodes vitamin K-dependent coagulation factor IX that circulates in the blood as an inactive zymogen. This factor is converted to an active form by factor XIa, which excises the activation peptide and thus generates a heavy chain and a light chain held together by one or more disulfide bonds. The role of this activated factor IX in the blood coagulation cascade is to activate factor X to its active form through interactions with Ca+2 ions, membrane phospholipids, and factor VIII. Alterations of this gene, including point mutations, insertions and deletions, cause factor IX deficiency, which is a recessive X-linked disorder, also called hemophilia B or Christmas disease. [provided by RefSeq]

Function: Factor IX is a vitamin K-dependent plasma protein that participates in the intrinsic pathway of blood coagulation by converting factor X to its active form in the presence of Ca2+ ions, phospholipids, and factor VIIIa. 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; disulfide-linked. Subcellular location: Secreted. Tissue specificity: Synthesized primarily in the liver and secreted in plasma. Domain: Calcium binds to the gamma-carboxyglutamic acid (Gla) residues and, with stronger affinity, to another site, beyond the Gla domain. Post-translational modification: Activated by factor XIa, which excises the activation peptide.The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. Involvement in disease: Defects in F9 are the cause of recessive X-linked hemophilia B (HEMB) [. MIM:306900]; also known as Christmas disease. Ref.11 Ref.13 Ref.34 Ref.37 Ref.38 Ref.39 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.62 Ref.63 Ref.64 Ref.65 Ref.66 Ref.67 Ref.70 Ref.71 Ref.72Note=Mutations in position 43 (Oxford-3, San Dimas) and 46 (Cambridge) prevents cleavage of the propeptide, mutation in position 93 (Alabama) probably fails to bind to cell membranes, mutation in position 191 (Chapel-Hill) or in position 226 (Nagoya OR Hilo) prevent cleavage of the activation peptide.Defects in F9 are the cause of thrombophilia due to factor IX defect (THR-FIX) [. MIM:300807]. A hemostatic disorder characterized by a tendency to thrombosis. Ref.73. Pharmaceutical use: Available under the name BeneFix (Baxter and American Home Products). Used to treat hemophilia B. Miscellaneous: In 1952, one of the earliest researchers of the disease, Dr. R.G. Macfarlane used the patient's surname, Christmas, to refer to the disease and also to refer to the clotting factor which he called the 'Christmas Factor' At the time Stephen Christmas was a 5-year-old boy. He died in 1993 at the age of 46 from acquired immunodeficiency syndrome contracted through treatment with blood products. 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

340 USD