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
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3.4.21.22
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hemophilia
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bleeding
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clot
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prothrombin
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thrombin
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thromboplastin
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anticoagulant
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platelet
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thrombosis
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antithrombin
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viiia
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x-linked
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heparin
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adeno-associated
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fibrinogen
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plasma-derived
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willebrand
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prophylaxis
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k-dependent
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infusions
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prophylactic
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hemostatic
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zymogen
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procoagulant
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haemostasis
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fixa
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thrombogenicity
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gamma-carboxyglutamic
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one-stage
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coagulopathy
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thromboembolic
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anti-factor
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amidolytic
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on-demand
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high-purity
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fibrinopeptide
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d-dimer
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thrombin-antithrombin
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exosite
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arthropathy
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prekallikrein
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cryoprecipitate
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medicine
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gamma-carboxylation
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rfviia
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bethesda
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alloantibodies
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prothrombinase
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thrombin-activated
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diathesis
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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
results (
results (Activating Compound
Activating Compound on EC 3.4.21.22 - coagulation factor IXa
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ACTIVATING COMPOUND 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
IMAGE
1,2-dicaproyl-sn-glycero-3-phospho-L-serine
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up-regulates the catalytic activity of the enzyme
1,2-propanediol
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25-40% v/v, up to 20fold increase of the activity, methoxycarbonyl-D-Nle-Gly-Arg-p-nitroanilide as substrate, rFIXa
emicizumab
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factorIXa/factorX bispecific antibody. Emicizumab functions as a cofactor to promote the factor IXa-catalyzed factor X activation
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ethanol
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25% v/v, 6fold increase of the activity, methoxycarbonyl-D-Nle-Gly-Arg-p-nitroanilide as substrate, rFIXa, alcohols modify the conformation of FIXa rendering the active-site cleft more easily accessible to tripeptide substrates with a hydrophobic residue in the P3-position
factor IX
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concentrations of approximately 100 nM, physiological levels in plasma, increases the activation of factor X catalyzed by factor IXa by increasing the affinity of the factor X for the intrinsic factor X activation complex
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factor IX-specific antibodies
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antibosies specific for human factor I, e.g. 198A1, 198B3, and 224F3, enhance the factor IXa activity mimicking the stimulatory effect of FVIIIa, and thus can replace FVIIIa as cofactor in the FX activation reaction, overview
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Factor XIa
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factor XIa proteolytically activates factor IX by an exosite- and Ca2+-mediated release-rebind mechanism. XIa cleaves IX after Arg145, forming IXalpha, and then after Arg180, forming IXabeta
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glycerol
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50% v/v, 6fold increase of the activity, methoxycarbonyl-D-Nle-Gly-Arg-p-nitroanilide as substrate, rFIXa
i-erythritol
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50% v/v, 1.5fold increase of the activity, methoxycarbonyl-D-Nle-Gly-Arg-p-nitroanilide as substrate, rFIXa
low molecular weight heparin
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enhances the activity of factor IXa (0.01 mM used in assay conditions)
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methanol
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25% v/v, 4fold increase of the activity, methoxycarbonyl-D-Nle-Gly-Arg-p-nitroanilide as substrate, rFIXa, alcohols modify the conformation of FIXa rendering the active-site cleft more easily accessible to tripeptide substrates with a hydrophobic residue in the P3-position
tert-butanol
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up to 10% v/v, increase of the activity, higher concentrations lead to a drastic activity decrease, methoxycarbonyl-D-Nle-Gly-Arg-p-nitroanilide as substrate, rFIXa, alcohols modify the conformation of FIXa rendering the active-site cleft more easily accessible to tripeptide substrates with a hydrophobic residue in the P3-position
TRYLRIHPQSWVHQIALRMEV
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approx. 600fold lower km, slight increase in kcat, optimal activation in the presence of 0.5-1 mM Ca2+
ethylene glycol
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25-40% v/v, up to 20fold increase of the activity, methoxycarbonyl-D-Nle-Gly-Arg-p-nitroanilide as substrate, rFIXa
ethylene glycol
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enhances the activity of factor IXa (30% (v/v) used in assay conditions)
Factor VIIIa
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protein cofactor, consists of A1, A2, and A3-C1-C2 subunits, A2 enhances the turnover number 100fold, the A1 subunit increases the A2-dependent stimulation by 10fold
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Factor VIIIa
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200000fold activation in the presence of phospholipid membrane through a combinantion of a decrease in Km and an increase in kcat
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Factor VIIIa
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a ternary complex of factor IXa, factor VIIIa and factor X is assembled on the activated platelet surface
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Factor VIIIa
stoichoimetric complex with factor IXa, helix 330 of factor IXa interacts with the 558-565 region of factor VIII A2 subunit
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Factor VIIIa
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the tenase complex, consisting of factor IXa and factor VIIIa assembled on a phospholipid-containing membrane, is the kinetically significant activator of factor X
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Factor VIIIa
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binding curves of FIXa and FVIIIa, binding of factor VIIIa with engineered disulfide bond resulting in a more stable FVIIIa: C662C1828 FVIIIa binds with similar affinity to that of wild-type FVIIIa, while C664-C1826 FVIIIa binds to FIXa with a significantly lower affinity, binding structures, overview
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Factor VIIIa
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binds to the activated factor IX and enhances the activation of factor X by several orders of magnitude
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Factor VIIIa
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factor IXa acts as part of phosphocholine/phosphoserine vesicles interacting with factor VIIIa in the intrinsic tenase complex
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Factor VIIIa
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factor VIIIa functions as a cofactor for factor IXa in the conversion of zymogen factor X to factor Xa. Factor IXa binding affinity for factor VIIIa is significantly reduced in the presence of GMA8011 (about 10fold)
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Factor VIIIa
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factor VIIIa may directly modulate the active catalytic site of factor IXa to increase kcat of the factor IXa-catalyzed factor X activation
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heparin
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activates by mediating cofactor binding, heparin binds to an exosite on the factor IXa protease domain, the factor IXa heparin-binding exosite participates in both cofactor binding and protease activation, and cofactor affinity is linked to active site conformation and factor X interaction during enzyme assembly, overview
Phospholipid
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the reaction is performed by the intrinsic FX-activating complex consisting of FIXa and FVIIIa on a negatively charged phospholipid surface in presence of Ca2+
phospholipid membrane
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200000fold activation in the presence of factor VIIIa through a combinantion of a decrease in Km and an increase in kcat
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phospholipid membrane
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phospholipid vesicles composed of 1-palmityl-2-oleoyl-sn-glycero-3-phosphoethanolamine, phosphatidylserine and phosphatidylcholine support factor Xa generation
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Phospholipids
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factor IXa acts as part of phosphocholine/phosphoserine vesicles interacting with factor VIIIa in the intrinsic tenase complex
Phospholipids
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the vesicles binding the enzyme consist of 40% phosphatidylcholine, 20% phosphatidylserine, and 40% phosphatidylethanolamine
