KEGG    Network variation - Coagulation cascade
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ENTRYnt06514
NameCoagulation cascade
CategoryPathway view; Immune system
Pathwayhsa04610 Complement and coagulation cascades
DiseaseH00219 Hemophilia
H00223 Inherited thrombophilia
H01006 Hereditary angioedema
Display drug-target relation   disease type
   D34,D35,D36
 |		 D38
 |
N01506    F12−Kallikrein→F12a
    FXII deficiency/HAE3 F12*
     D38
 |		  
N01507      F11−F12a→F11a
    FXI deficiency   F11*
         D24
 |		 D24
 |
N01508        F9−F11a→F9a→(F9a+F8a)
     D17,D18,D19,D20,D21,D22,D23
 |
    HEMB/THPH8     F9*
     D2
 |		 D27,D31,D32,D33,D29,D30
 |		   D24
 |
N01509        (F8+VWF)−(F10a,F2a)→F8a→(F9a+F8a)
     D3,D4,D5,D6,D7,D8,D9,D10,D11,D12,D13,D14,D15,D16
 |
    HEMA     F8*
     D5
 |
    VWD     VWF*
 
   D24,D27,D31,D32,D33,D38,D29,D30,D28
 |		 D37,D25
 |		 D37,D25
 |
N01503    F7−Protease→F7a→(F7a+F3)
    FVII deficiency/MI F7*
 
 D24,D25
 |		 D24,D37,D25
 |		 D27,D31,D32,D33
 |		 D27,D31,D32,D33
 |
N01510    F10−((F9a+F8a),(F7a+F3..→F10a→(F10a+F5a)
    FX deficiency F10*
   D29,D30
 |		   D27,D31,D32,D33
 |
N01511    F5−F2a→F5a→(F10a+F5a)
    FV deficiency/THPH2/BDCHS F5*
       D27,D31,D32,D33
 |		 D29,D30
 |
N01512        F2−(F10a+F5a)→F2a
    FII deficiency/THPH1     F2*
         D29,D30
 |		  
N01513          FG−F2a→Fibrin
    Afibrinogenemia/Amyloidosis       FGA*
    Afibrinogenemia       FGB*
    Afibrinogenemia       FGG*
         D29,D30
 |		  
N01514          F13−F2a→F13a
    FXIIIA deficiency/MI       F13A1*
    FXIIIB deficiency       F13B*
         D29,D30
 |
N01521          SERPIND1⊣F2a
    THPH10       SERPIND1*
 
     D28
 |
N01518    PLG−(PLAU,PLAT)→Plasmin
    PLG deficiency/HAE4 PLG*
    QPD   PLAU*↗
N01524    PAI⊣(PLAU,PLAT)
    PAI-1 deficiency SERPINE1*
     D28
 |
N01522      SERPINF2⊣Plasmin
    a2-PI deficiency   SERPINF2*
     D28
 |
N01523      SERPINA1⊣(Plasmin,PLAU)
    A1AT deficiency/COPD   SERPINA1*
 
   D29,D30
 |		      
N01515    PROC−(F2a+THBD+PROCR)→APC→(APC+PROS1)⊣(F5a,F8a)
    THPH3/4 PROC*
    THPH12   THBD*
    THPH5/6       PROS1*
 
 D26
 |		 D38
 |		 D34,D35,D36
 |
N01516    KLKB1−F12a→Kallikrein
    PKKD KLKB1*
     D34,D35,D36
 |		    
N01517      KNG1−Kallikrein→Bradykinin→BDKRB
    HAE6   KNG1*
 
   D38,D34,D35,D36,D28
 |
N01520    SERPING1⊣(F11a,F12a,Kallikr..
    C1NH deficiency/HAE1/2 SERPING1*
 D27
 |		 D29,D30,D24,D27,D31,D32,D33,D38
 |
N01519    SERPINC1⊣(F2a,F9a,F10a,F11a..
    AT deficiency/THPH7 SERPINC1*
 
 D1
 |		    
N01831    ADAMTS13⊣VWF→(GP1BA+GP1BB+GP9+G..
    TTP ADAMTS13*
    BSS/PT-VWD     GP1BA*
    BSS     GP1BB*
    BSS     GP9*

Disease nameDisease category
FXII deficiency/HAE3H00941Factor XII deficiencyHematologic disease
H01006Hereditary angioedemaCardiovascular disease
FXI deficiencyH00938Factor XI deficiencyHematologic disease
HEMB/THPH8H00219HemophiliaHematologic disease
H00223Inherited thrombophiliaHematologic disease
HEMAH00219HemophiliaHematologic disease
VWDH02092von Willebrand diseaseHematologic disease
H00219HemophiliaHematologic disease
FVII deficiency/MIH02256Factor VII deficiencyHematologic disease
H01730Myocardial infarctionCardiovascular disease
FX deficiencyH02257Factor X deficiencyHematologic disease
FV deficiency/THPH2/BDCHSH00220Factor V deficiencyHematologic disease
H00223Inherited thrombophiliaHematologic disease
H01433Budd-Chiari syndromeDigestive system disease
FII deficiency/THPH1H01254Congenital prothrombin deficiencyHematologic disease
H00223Inherited thrombophiliaHematologic disease
Afibrinogenemia/AmyloidosisH00222Congenital fibrinogen deficiencyHematologic disease
H00845Familial amyloidosisNervous system disease
AfibrinogenemiaH00222Congenital fibrinogen deficiencyHematologic disease
FXIIIA deficiency/MIH00945Factor XIII deficiencyHematologic disease
H01730Myocardial infarctionCardiovascular disease
FXIIIB deficiencyH00945Factor XIII deficiencyHematologic disease
THPH10H00223Inherited thrombophiliaHematologic disease
PLG deficiency/HAE4H01206Plasminogen deficiencyHematologic disease
H01006Hereditary angioedemaCardiovascular disease
QPDH01235Bleeding disorder platelet-typeHematologic disease
PAI-1 deficiencyH01106Plasminogen activator inhibitor type 1 deficiencyHematologic disease
a2-PI deficiencyH00983Alpha-2-plasmin inhibitor (a2-PI) deficiencyHematologic disease
A1AT deficiency/COPDH01103Alpha-1-antitrypsin deficiencyInherited metabolic disorder
H01714Chronic obstructive pulmonary disease (COPD)Respiratory system disease
THPH3/4H00223Inherited thrombophiliaHematologic disease
THPH12H00223Inherited thrombophiliaHematologic disease
THPH5/6H00223Inherited thrombophiliaHematologic disease
PKKDH01078Fletcher factor deficiencyHematologic disease
HAE6H01006Hereditary angioedemaCardiovascular disease
C1NH deficiency/HAE1/2H00106Complement regulatory protein defectsPrimary immunodeficiency
H01006Hereditary angioedemaCardiovascular disease
AT deficiency/THPH7H01381Antithrombin III deficiencyHematologic disease
H00223Inherited thrombophiliaHematologic disease
TTPH00225Thrombotic thrombocytopenic purpuraCardiovascular disease
BSS/PT-VWDH01740MacrothrombocytopeniaHematologic disease
H02093Platelet-type von Willebrand diseaseHematologic disease
BSSH01740MacrothrombocytopeniaHematologic disease


Drug name
D1D12835Apadamtase alfa and cinaxadamtase alfa
D2D02235Desmopressin acetate (USP)
D3D05409Rurioctocog alfa (genetical recombination) (JAN)
D4D08232Moroctocog alfa (INN)
D5D08798Freeze-dried human blood-coagulation factor VIII
D6D10227Turoctocog alfa (INN)
D7D10539Efraloctocog alfa (genetical recombination) (JAN)
D8D10758Turoctocog alfa pegol (genetical recombination) (JAN)
D9D10759Damoctocog alfa pegol (INN)
D10D10760Rurioctocog alfa pegol (genetical recombination) (JAN)
D11D10771Octocog beta (genetical recombination) (JAN)
D12D10818Lonoctocog alfa (INN)
D13D10830Efmoroctocog alfa (INN)
D14D10831Susoctocog alfa (INN)
D15D10842Simoctocog alfa (INN)
D16D12441Efanesoctocog alfa (INN)
D17D05201Nonacog alfa (USAN/INN)
D18D08794Human blood-coagulation factor IX complex, dried
D19D08797Freeze-dried human blood-coagulation factor IX
D20D10522Eftrenonacog alfa (INN)
D21D10757Nonacog beta pegol (INN)
D22D10770Albutrepenonacog alfa (INN)
D23D12908Fidanacogene elaparvovec (USAN/INN)
D24D10821Emicizumab (USAN/INN)
D25D12421Freeze-dried activated human blood coagulation factor VII concentrate containing factor X
D26D13154Donidalorsen sodium (USAN)
D27D01844Fondaparinux sodium (JAN/USP/INN)
D28D01136Tranexamic acid (JP18/USP/INN)
D29D00181Argatroban (USP)
D30D07082Dabigatran etexilate mesylate (USAN)
D31D03213Apixaban (JAN/USAN/INN)
D32D07086Rivaroxaban (JAN/USAN/INN)
D33D09546Edoxaban tosilate hydrate (JAN)
D34D03931Ecallantide (USAN/INN)
D35D11094Lanadelumab (USAN)
D36D11674Berotralstat hydrochloride (JAN/USAN)
D37D00172Eptacog alfa (INN)
D38D12613Garadacimab (INN)