catalog number :
MBS142908
products type :
Native Protein
products full name :
Human Coagulation Factor IX
products short name :
Coagulation Factor IX
products name syn :
F9 Human; Coagulation Factor IX Human; Coagulation factor IX; EC 3.4.21.22; Christmas factor; Plasma thromboplastin component; PTC; F9; FIX; HEMB; MGC129641; MGC129642; GLA domain; Factor IX
other names :
coagulation factor IX preproprotein; Coagulation factor IX; coagulation factor IX; Christmas factor; F9 p22; FIX F9; factor 9; factor IX F9; plasma thromboplastic component; plasma thromboplastin component; coagulation factor IX; Christmas factor; Plasma thromboplastin component; PTC
other gene names :
F9; F9; FIX; P19; PTC; HEMB; THPH8; PTC
uniprot entry name :
FA9_HUMAN
purity :
Greater than 95.0% as determined by SDS-PAGE.
form :
The Factor-IX was lyophilized from a sterile solution containing 20mM Tris-HCl pH-7.4, 0.1M NaCl and 1mM Benzamidine. Sterile Filtered White lyophilized (freeze-dried) powder.
storage stability :
Lyophilized Factor-IX although stable at room temperature for 3 weeks, should be stored desiccated below -18 degree C. Upon reconstitution Factor-IX should be stored at 4 degree C between 2-7 days and for future use below -18 degree C.Please prevent freeze-thaw cycles.
other info2 :
Solubility: It is recommended to reconstitute the lyophilized 100U Factor-IX in sterile 100 ul of 18M Omega -cm H2O, which can then be further diluted to other aqueous solutions. Human Virus Test: Human plasma was tested and found negative for HIV-1, HIV-2, Hepatitis B Surface antigen and HCV. Donors are screened for CJD (Creutzfeldt-Jakob Disease). Biological Activity: The activity per mg was tested and found to be 230 PEU/mg.
products categories :
RECOMBINANT & NATURAL PROTEINS; Natural Proteins; Coagulation Factors
products description :
Description: Human Factor-IX produced from fresh frozen human plasma is a glycosylated polypeptide chain having a molecular mass of 56 kDa. Introduction: Human Factor IX also called Christmas-Factor is a glycoprotein, which is synthesized in the liver and belongs to the serine proteases system and is part of the S1 peptidase family.Lack of Factor-IX causes Hemophilia-B meaning Christmas Disease. Factor-IX has a N-terminus region which contains 12xGla residues which asist the calcium dpendant binding of Factor-IX to the phospholipid surface. Factor-IX is activated by either factor XIa or the factor VIIa/tissue factor/phospholipid complex. Cleavage yields the intermediate IXa, which is subsequently converted to the fully active form IXab.Factor-IX binds initially to exosites on the factor XIa heavy chain, followed by interaction at the active site with subsequent bond cleavage. Coagulation factor IX is activated by interaction with the erythrocyte membrane, causing intrinsic coagulation. Chaperones & lectins act simultaniously to guarantee the proper folding of Factor-IX and the retention of mutant molecules. Human Factor IX, activated by either the Contact or Tissue Factor Pathway, is responsible for the activation of Factor X to Xa.
ncbi acc num :
NP_000124.1
ncbi gb acc num :
NM_000133.3
ncbi mol weight :
47,618 Da
ncbi pathways :
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, Hypusine Formation And Arylsulfatase Activation Pathway (106231); Gamma-carboxylation Of Protein Precursors Pathway (106233); Gamma-carboxylation, Transport, And Amino-terminal Cleavage Of Proteins Pathway (106232); Hemostasis Pathway (106028)
ncbi summary :
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, Jul 2008]
uniprot summary :
F9: 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 Ca(2+) ions, phospholipids, and factor VIIIa. Defects in F9 are the cause of recessive X-linked hemophilia B (HEMB); also known as Christmas disease. 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 (THPH8). A hemostatic disorder characterized by a tendency to thrombosis. Belongs to the peptidase S1 family. Protein type: Protease; Secreted; Secreted, signal peptide; EC 3.4.21.22. Chromosomal Location of Human Ortholog: Xq27.1-q27.2. Cellular Component: Golgi lumen; endoplasmic reticulum lumen; plasma membrane; extracellular region. Molecular Function: serine-type endopeptidase activity; calcium ion binding. Biological Process: blood coagulation, extrinsic pathway; cellular protein metabolic process; proteolysis; blood coagulation; post-translational protein modification; blood coagulation, intrinsic pathway; peptidyl-glutamic acid carboxylation. Disease: Hemophilia B; Thrombophilia, X-linked, Due To Factor Ix Defect; Coumarin Resistance