3.4.21.22: coagulation factor IXa
This is an abbreviated version!
For detailed information about coagulation factor IXa, go to the full flat file.
Word Map on EC 3.4.21.22
-
3.4.21.22
-
hemophilia
-
bleeding
-
clot
-
prothrombin
-
thrombin
-
thromboplastin
-
anticoagulant
-
platelet
-
thrombosis
-
antithrombin
-
viiia
-
x-linked
-
heparin
-
adeno-associated
-
fibrinogen
-
plasma-derived
-
willebrand
-
prophylaxis
-
k-dependent
-
infusions
-
prophylactic
-
hemostatic
-
zymogen
-
procoagulant
-
haemostasis
-
fixa
-
thrombogenicity
-
gamma-carboxyglutamic
-
one-stage
-
coagulopathy
-
thromboembolic
-
anti-factor
-
amidolytic
-
on-demand
-
high-purity
-
fibrinopeptide
-
d-dimer
-
thrombin-antithrombin
-
exosite
-
arthropathy
-
prekallikrein
-
cryoprecipitate
-
medicine
-
gamma-carboxylation
-
rfviia
-
bethesda
-
alloantibodies
-
prothrombinase
-
thrombin-activated
-
diathesis
-
novoseven
- 3.4.21.22
- hemophilia
- bleeding
- clot
- prothrombin
- thrombin
- thromboplastin
-
anticoagulant
- platelet
- thrombosis
- antithrombin
- viiia
-
x-linked
- heparin
-
adeno-associated
- fibrinogen
-
plasma-derived
- willebrand
-
prophylaxis
-
k-dependent
-
infusions
-
prophylactic
-
hemostatic
- zymogen
-
procoagulant
-
haemostasis
- fixa
-
thrombogenicity
-
gamma-carboxyglutamic
-
one-stage
- coagulopathy
-
thromboembolic
-
anti-factor
-
amidolytic
-
on-demand
-
high-purity
-
fibrinopeptide
-
d-dimer
-
thrombin-antithrombin
-
exosite
- arthropathy
- prekallikrein
-
cryoprecipitate
- medicine
-
gamma-carboxylation
- rfviia
-
bethesda
-
alloantibodies
- prothrombinase
-
thrombin-activated
- diathesis
- novoseven
Reaction
Selective cleavage of Arg-/-Ile bond in factor X to form factor Xa =
Synonyms
activated Christmas factor, activated coagulation factor IX, activated factor IX, activated FIX, blood coagulation factor IXa, Christmas factor, circulating factor IXa, coagulation factor IX, coagulation factor IXa, factor IX, factor IXa, factor IXaAL, factor IXaalpha, factor IXabeta, factor IXabeta', factor IXaCH, factor IXalphabeta, factor IXaN, factor XIa, FIX, FIXa, FIXC, Gla-domainless factor IXabeta', human coagulation factor IXa, intrinsic Xase, More
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3.4.21.22 coagulation factor IXaAdvanced search results
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Reference on EC 3.4.21.22 - coagulation factor IXa
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Fujikawa, K.; Legaz, M.E.; Kato, H.; Davie, E.W.
The mechanism of activation of bovine factor IX (Christmas factor) by bovine factor XIa (activated plasma thromboplastin antecedent)
Biochemistry
13
4508-4516
1974
Bos taurus
Thompson, A.R.
Structure, function and molecular defects of factor IX
Blood
67
565-572
1986
Homo sapiens
Van Dieijen, G.; Janssen-Claessen, T.
Purification of coagulation factors and preparation of reagents for chromogenic substrate assays
Methods Enzym. Anal. , 3rd Ed. (Bergmeyer, H. U. , ed. )
5
390-394
1986
Bos taurus
-
Osterud, B.; Bouma, B.N.; Griffin, J.H.
Human blood coagulation factor IX
J. Biol. Chem.
253
5946-5951
1978
Homo sapiens
Sturzebecher, J.; Kopetzki, E.; Bode, W.; Hopfner, K.P.
Dramatic enhancement of the catalytic activity of coagulation factor IXa by alcohols
FEBS Lett.
412
295-300
1997
Homo sapiens
Morita, T.; Fukudome, K.; Miyata, T.; Iwanaga, S.
gamma-Carboxylglutamic acid (Gla)-domainless blood coagulation factor IXa species: preparation and properties
J. Biochem.
110
990-996
1991
Bos taurus
London, F.S.; Walsh, P.N.
Zymogen factor IX potentiates factor IXa-catalyzed activation
Biochemistry
39
9850-9858
2000
Homo sapiens
Hopfner, K.P.; Lang, A.; Karcher, A.; Sichler, K.; Kopetzki, E.; Barndstetter, H.; Huber, R.; Bode, W.; Engh, R.A.
Coagulation factor IXa: the relaxed conformation of Tyr99 blocks substrate binding
Structure Fold. Des.
7
989-996
1999
Homo sapiens
Fay, P.J.; Koshibu, K.; Mastri, M.
The A1 and the A2 subunits of factor VIIIa synergistically stimulate factor IXa catalytic activity
J. Biol. Chem.
274
15401-15406
1999
Homo sapiens
Ahmed, S.S.; Rawala-Sheikh, R.; Monroe, D.M.; Roberts, H.R.; Walsh, P.N.
Comparative platelet binding and kinetic studies with normal and variant factor IXa molecules
J. Biol. Chem.
265
20907-20911
1990
Homo sapiens
Blostein, M.D.; Rigby, A.C.; Furie, B.C.; Furie, B.; Gilbert, G.E.
Amphipathic helices support function of blood coagulation factor IXa
Biochemistry
39
12000-12006
2000
Homo sapiens
Falls, L.A.; Furie, B.; Furie, B.C.
Role of phosphatidylethanolamine in assembly and function of the factor IXa-factor VIIIa complex on membrane surfaces
Biochemistry
39
13216-13222
2000
Homo sapiens
Ahmad, S.S.; Walsh, P.N.
Coordinate binding studies of the substrate (factor X) with the cofactor (factor VIII) in the assembly of the factor X activating complex on the activated platelet surface
Biochemistry
41
11269-11276
2002
Homo sapiens
Bedsted, T.; Swanson, R.; Chuang, Y.J.; Bock, P.E.; Bjork, I.; Olson, S.T.
Heparin and calcium ions dramatically enhance antithrombin reactivity with factor IXa by generating new interaction exosites
Biochemistry
42
8143-8152
2003
Homo sapiens
Bajaj, S.P.; Schmidt, A.E.; Mathur, A.; Padmanabhan, K.; Zhong, D.; Mastri, M.; Fay, P.J.
Factor IXa:factor VIIIa interaction. helix 330-338 of factor ixa interacts with residues 558-565 and spatially adjacent regions of the a2 subunit of factor VIIIa
J. Biol. Chem.
276
16302-16309
2001
Homo sapiens (P00740)
Wilkinson, F.H.; London, F.S.; Walsh, P.N.
Residues 88-109 of factor IXa are important for assembly of the factor X activating complex
J. Biol. Chem.
277
5725-5733
2002
Homo sapiens
Yang, L.; Manithody, C.; Olson, S.T.; Rezaie, A.R.
Contribution of basic residues of the autolysis loop to the substrate and inhibitor specificity of factor IXa
J. Biol. Chem.
278
25032-25038
2003
Homo sapiens (P00740)
Blostein, M.D.; Furie, B.C.; Rajotte, I.; Furie, B.
The Gla domain of factor IXa binds to factor VIIIa in the tenase complex
J. Biol. Chem.
278
31297-31302
2003
Homo sapiens
Wiebe, E.M.; Stafford, A.R.; Fredenburgh, J.C.; Weitz, J.I.
Mechanism of catalysis of inhibition of factor IXa by antithrombin in the presence of heparin or pentasaccharide
J. Biol. Chem.
278
35767-35774
2003
Homo sapiens
Rohlena, J.; Kolkman, J.A.; Boertjes, R.C.; Mertens, K.; Lenting, P.J.
Residues Phe342-Asn346 of activated coagulation factor IX contribute to the interaction with low density lipoprotein receptor-related protein
J. Biol. Chem.
278
9394-9401
2003
Homo sapiens
Rusconi, C.P.; Scardino, E.; Layzer, J.; Pitoc, G.A.; Ortel, T.L.; Monroe, D.; Sullenger, B.A.
RNA aptamers as reversible antagonists of coagulation factor IXa
Nature
419
90-94
2002
Homo sapiens
Panteleev, M.A.; Saenko, E.L.; Ananyeva, N.M.; Ataullakhanov, F.I.
Kinetics of Factor X activation by the membrane-bound complex of Factor IXa and Factor VIIIa
Biochem. J.
381
779-794
2004
Homo sapiens
Yang, X.; Walsh, P.N.
An ordered sequential mechanism for Factor IX and Factor IXa binding to platelet receptors in the assembly of the Factor X-activating complex
Biochem. J.
390
157-167
2005
Homo sapiens
Neuenschwander, P.F.
Exosite occupation by heparin enhances the reactivity of blood coagulation factor IXa
Biochemistry
43
2978-2986
2004
Homo sapiens
Batt, D.G.; Qiao, J.X.; Modi, D.P.; Houghton, G.C.; Pierson, D.A.; Rossi, K.A.; Luettgen, J.M.; Knabb, R.M.; Jadhav, P.K.; Wexler, R.R.
5-Amidinoindoles as dual inhibitors of coagulation factors IXa and Xa
Bioorg. Med. Chem. Lett.
14
5269-5273
2004
Homo sapiens
Qiao, J.X.; Cheng, X.; Modi, D.P.; Rossi, K.A.; Luettgen, J.M.; Knabb, R.M.; Jadhav, P.K.; Wexler, R.R.
5-Amidinobenzo[b]thiophenes as dual inhibitors of factors IXa and Xa
Bioorg. Med. Chem. Lett.
15
29-35
2005
Homo sapiens
Lu, G.; Broze, G.J.; Krishnaswamy, S.
Formation of factors IXa and Xa by the extrinsic pathway: differential regulation by tissue factor pathway inhibitor and antithrombin III
J. Biol. Chem.
279
17241-17249
2004
Homo sapiens
Butenas, S.; Orfeo, T.; Gissel, M.T.; Brummel, K.E.; Mann, K.G.
The significance of circulating factor IXa in blood
J. Biol. Chem.
279
22875-22882
2004
Homo sapiens
Yang, X.; Chang, Y.J.; Lin, S.W.; Walsh, P.N.
Identification of residues Asn89, Ile90, and Val107 of the factor IXa second epidermal growth factor domain that are essential for the assembly of the factor X-activating complex on activated platelets
J. Biol. Chem.
279
46400-46405
2004
Homo sapiens (P00740)
Schmidt, A.E.; Stewart, J.E.; Mathur, A.; Krishnaswamy, S.; Bajaj, S.P.
Na+ site in blood coagulation factor IXa: effect on catalysis and factor VIIIa binding
J. Mol. Biol.
350
78-91
2005
Homo sapiens (P00740)
Ndonwi, M.; Broze, G.; Bajaj, S.P.
The first epidermal growth factor-like domains of factor Xa and factor IXa are important for the activation of the factor VII-tissue factor complex
J. Thromb. Haemost.
3
112-118
2005
Homo sapiens
London, F.S.; Marcinkiewicz, M.; Walsh, P.N.
PAR-1-stimulated factor IXa binding to a small platelet subpopulation requires a pronounced and sustained increase of cytoplasmic calcium
Biochemistry
45
7289-7298
2006
Homo sapiens
Misenheimer, T.M.; Buyue, Y.; Sheehan, J.P.
The heparin-binding exosite is critical to allosteric activation of factor IXa in the intrinsic tenase complex: the role of arginine 165 and factor X
Biochemistry
46
7886-7895
2007
Homo sapiens
Neuenschwander, P.F.; Williamson, S.R.; Nalian, A.; Baker-Deadmond, K.J.
Heparin modulates the 99-loop of factor IXa: effects on reactivity with isolated Kunitz-type inhibitor domains
J. Biol. Chem.
281
23066-23074
2006
Homo sapiens (P00740)
Gale, A.J.; Radtke, K.P.; Cunningham, M.A.; Chamberlain, D.; Pellequer, J.L.; Griffin, J.H.
Intrinsic stability and functional properties of disulfide bond-stabilized coagulation factor VIIIa variants
J. Thromb. Haemost.
4
1315-1322
2006
Homo sapiens
Scheiflinger, F.; Dockal, M.; Rosing, J.; Kerschbaumer, R.J.
Enhancement of the enzymatic activity of activated coagulation factor IX by anti-factor IX antibodies
J. Thromb. Haemost.
6
315-322
2008
Homo sapiens
Yang, L.; Gopalakrishna, K.; Manithody, C.; Rezaie, A.R.
Expression, purification and characterization of factor IX derivatives using a novel vector system
Protein Expr. Purif.
50
196-202
2006
Homo sapiens (P00740)
Gopalakrishna, K.; Rezaie, A.R.
The influence of sodium ion binding on factor IXa activity
Thromb. Haemost.
95
936-941
2006
Homo sapiens
Zoegg, T.; Brandstetter, H.
Activation mechanisms of coagulation factor IX
Biol. Chem.
390
391-400
2009
Homo sapiens
Yang, Y.H.; Chien, D.; Wu, M.; FitzGerald, J.; Grossman, J.M.; Hahn, B.H.; Hwang, K.K.; Chen, P.P.
Novel autoantibodies against the activated coagulation factor IX (FIXa) in the antiphospholipid syndrome that interpose the FIXa regulation by antithrombin
J. Immunol.
182
1674-1680
2009
Homo sapiens
Becker, R.C.; Oney, S.; Becker, K.C.; Sullenger, B.
Antidote-controlled antithrombotic therapy targeting factor IXa and von Willebrand factor
Ann. N. Y. Acad. Sci.
1175
61-70
2009
Homo sapiens
Gettins, P.G.; Olson, S.T.
Activation of antithrombin as a factor IXa and Xa inhibitor involves mitigation of repression rather than positive enhancement
FEBS Lett.
583
3397-3400
2009
Homo sapiens
Kim, W.H.; Kim, J.S.; Kim, J.S.; Yoon, Y.; Lee, G.M.
Effect of Ca2+ and Mg2+ concentration in culture medium on the activation of recombinant factor IX produced in Chinese hamster ovary cells
J. Biotechnol.
142
275-278
2009
Homo sapiens
Hartmann, R.; Dockal, M.; Kammlander, W.; Panholzer, E.; Nicolaes, G.A.; Fiedler, C.; Rosing, J.; Scheiflinger, F.
Factor IX mutants with enhanced catalytic activity
J. Thromb. Haemost.
7
1656-1662
2009
Homo sapiens
Zögg, T.; Brandstetter, H.
Structural basis of the cofactor- and substrate-assisted activation of human coagulation factor IXa
Structure
17
1669-1678
2009
Homo sapiens (P00740), Homo sapiens
Hosseini, S.; Zomorodipour, A.; Jalal, R.; Sabouni, F.; Ataei, F.
A study of the expression of functional human coagulation factor IX in keratinocytes using a nonviral vector regulated by K14 promoter
Appl. Biochem. Biotechnol.
162
1599-1611
2010
Homo sapiens
Misenheimer, T.M.; Sheehan, J.P.
The regulation of factor IXa by supersulfated low molecular weight heparin
Biochemistry
49
9997-10005
2010
Homo sapiens
Yang, L.; Manithody, C.; Qureshi, S.H.; Rezaie, A.R.
Role of the residues of the 39-loop in determining the substrate and inhibitor specificity of factor IXa
J. Biol. Chem.
285
28488-28495
2010
Homo sapiens
Wang, S.; Beck, R.; Blench, T.; Burd, A.; Buxton, S.; Malic, M.; Ayele, T.; Shaikh, S.; Chahwala, S.; Chander, C.; Holland, R.; Merette, S.; Zhao, L.; Blackney, M.; Watts, A.
Studies of benzothiophene template as potent factor IXa (FIXa) inhibitors in thrombosis
J. Med. Chem.
53
1465-1472
2010
Homo sapiens
Wang, S.; Beck, R.; Burd, A.; Blench, T.; Marlin, F.; Ayele, T.; Buxton, S.; Dagostin, C.; Malic, M.; Joshi, R.; Barry, J.; Sajad, M.; Cheung, C.; Shaikh, S.; Chahwala, S.; Chander, C.; Baumgartner, C.; Holthoff, H.P.; Murray, E.; Blackney, M.; Giddings, A.
Structure based drug design: development of potent and selective factor IXa (FIXa) inhibitors
J. Med. Chem.
53
1473-1482
2010
Homo sapiens
Chang, L.; Yang, C.; Chua, A.; Lin, Y.; Lai, S.
Sustained release of transgenic human factor IX: Preparation, characterization, and in vivo efficacy
Mol. Pharm.
8
1767-1774
2011
Homo sapiens
Johnson, D.J.; Langdown, J.; Huntington, J.A.
Molecular basis of factor IXa recognition by heparin-activated antithrombin revealed by a 1.7-A structure of the ternary complex
Proc. Natl. Acad. Sci. USA
107
645-650
2010
Homo sapiens (P00740)
Wu, H.; Xu, X.; Zhang, L.; Shen, D.; Peng, L.; Zhang, Y.; Song, J.
Mg(II)-induced binding of factor IX-binding protein from the venom of Agkistrodon Halys Pallas with factor Xa
Toxicon
55
1358-1364
2010
Homo sapiens
Cunha, N.; Murad, A.; Ramos, G.; Maranhao, A.; Brígido, M.; Araujo, A.; Lacorte, C.; Aragao, F.; Covas, D.; Fontes, A.; Souza, G.; Vianna, G.; Rech, E.
Accumulation of functional recombinant human coagulation factor IX in transgenic soybean seeds
Transgenic Res.
20
841-855
2011
Homo sapiens (P00740), Homo sapiens
Venkateswarlu, D.
Structural insights into the interaction of blood coagulation co-factor VIIIa with factor IXa: a computational protein-protein docking and molecular dynamics refinement study
Biochem. Biophys. Res. Commun.
452
408-414
2014
Homo sapiens
Branchini, A.; Campioni, M.; Mazzucconi, M.; Biondo, F.; Mari, R.; Bicocchi, M.; Bernardi, F.; Pinotti, M.
Replacement of the Y450 (c234) phenyl ring in the carboxyl-terminal region of coagulation factor IX causes pleiotropic effects on secretion and enzyme activity
FEBS Lett.
587
3249-3253
2013
Homo sapiens
Sullenger, B.; Woodruff, R.; Monroe, D.M.
Potent anticoagulant aptamer directed against factor IXa blocks macromolecular substrate interaction
J. Biol. Chem.
287
12779-12786
2012
Homo sapiens
Geng, Y.; Verhamme, I.M.; Messer, A.; Sun, M.F.; Smith, S.B.; Bajaj, S.P.; Gailani, D.
A sequential mechanism for exosite-mediated factor IX activation by factor XIa
J. Biol. Chem.
287
38200-38209
2012
Homo sapiens
Yang, L.; Rezaie, A.R.
Residues of the 39-loop restrict the plasma inhibitor specificity of factor IXa
J. Biol. Chem.
288
12692-12698
2013
Bos taurus, Mus musculus
Bloem, E.; Meems, H.; van den Biggelaar, M.; Mertens, K.; Meijer, A.B.
A3 domain region 1803-1818 contributes to the stability of activated factor VIII and includes a binding site for activated factor IX
J. Biol. Chem.
288
26105-26111
2013
Homo sapiens
Wakabayashi, H.; Fay, P.J.
Replacing the factor VIII C1 domain with a second C2 domain reduces factor VIII stability and affinity for factor IXa
J. Biol. Chem.
288
31289-31297
2013
Homo sapiens
Neuenschwander, P.F.; Deadmond, K.J.; Zepeda, K.; Rutland, J.
Correlation of factor IXa subsite modulations with effects on substrate discrimination
J. Thromb. Haemost.
10
382-389
2012
Homo sapiens
Majumder, R.; Koklic, T.; Sengupta, T.; Cole, D.; Chattopadhyay, R.; Biswas, S.; Monroe, D.; Lentz, B.R.
Soluble phosphatidylserine binds to two sites on human factor IXa in a Ca2+ dependent fashion to specifically regulate structure and activity
PLoS ONE
9
e100006
2014
Homo sapiens
Kristensen, L.H.; Olsen, O.H.; Blouse, G.E.; Brandstetter, H.
Releasing the brakes in coagulation factor IXa by co-operative maturation of the substrate-binding site
Biochem. J.
473
2395-2411
2016
Homo sapiens (P00740)
Meng, D.; Andre, P.; Bateman, T.J.; Berger, R.; Chen, Y.H.; Desai, K.; Dewnani, S.; Ellsworth, K.; Feng, D.; Geissler, W.M.; Guo, L.; Hruza, A.; Jian, T.; Li, H.; Metzger, J.; Parker, D.L.; Reichert, P.; Sherer, E.C.; Smith, C.J.; Sonatore, L.M.; Tschirret-Guth, R.; Wu, J.; Xu, J.; Zhang, T.; Campeau, L.C.; Orr,
Development of a novel tricyclic class of potent and selective FIXa inhibitors
Bioorg. Med. Chem. Lett.
25
5437-5443
2015
Homo sapiens
Sakurada, I.; Endo, T.; Hikita, K.; Hirabayashi, T.; Hosaka, Y.; Kato, Y.; Maeda, Y.; Matsumoto, S.; Mizuno, T.; Nagasue, H.; Nishimura, T.; Shimada, S.; Shinozaki, M.; Taguchi, K.; Takeuchi, K.; Yokoyama, T.; Hruza, A.; Reichert, P.; Zhang, T.; Wood, H.B.; Nakao, K.; Furusako, S.
Discovery of novel aminobenzisoxazole derivatives as orally available factor IXa inhibitors
Bioorg. Med. Chem. Lett.
27
2622-2628
2017
Homo sapiens (P00740)
Kitazawa, T.; Shima, M.
Emicizumab, a humanized bispecific antibody to coagulation factors IXa and X with a factor VIIIa-cofactor activity
Int. J. Hematol.
111
20-30
2018
Homo sapiens
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