KEGG   PATHWAY: mjv04610
Entry
mjv04610                    Pathway                                
Name
Complement and coagulation cascades - Manis javanica (Malayan pangolin)
Description
The complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation: the classical pathway, the lectin pathway, and the alternative pathway. All of these pathways generate a crucial enzymatic activity that, in turn, generates the effector molecules of complement. The main consequences of complement activation are the opsonization of pathogens, the recruitment of inflammatory and immunocompetent cells, and the direct killing of pathogens. Blood coagulation is another series of proenzyme-to-serine protease conversions, culminating the formation of thrombin, the enzyme responsible for the conversion of soluble fibrinogen to the insoluble fibrin clot. Protease-activated receptors, such as those activated by thrombin, are members of G protein-coupled receptors and function as a mediator of innate immunity. The kallikrein-kinin system is an endogenous metabolic cascade, triggering of which results in the release of vasoactive kinins (bradykinin-related peptides). Kinin peptides are implicated in many physiological and pathological processes including the regulation of blood pressure and sodium homeostasis, inflammatory processes, and the cardioprotective effects of preconditioning.
Class
Organismal Systems; Immune system
Pathway map
mjv04610  Complement and coagulation cascades
mjv04610

Organism
Manis javanica (Malayan pangolin) [GN:mjv]
Gene
108383641  F2R; proteinase-activated receptor 1 isoform X2 [KO:K03914]
108383644  F2RL2; proteinase-activated receptor 3 [KO:K04235]
108384291  SERPINE2; glia-derived nexin [KO:K16643]
108384823  [KO:K01330] [EC:3.4.21.41]
108384826  C1S; complement C1s subcomponent isoform X1 [KO:K01331] [EC:3.4.21.42]
108385629  [KO:K03910]
108385678  KNG1; kininogen-1 isoform X1 [KO:K03898]
108385786  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
108385846  MBL2; mannose-binding protein C [KO:K03991]
108385940  CD59; CD59 glycoprotein isoform X2 [KO:K04008]
108386638  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
108386642  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
108386643  [KO:K04002]
108388833  ITGB2; integrin beta-2 [KO:K06464]
108389700  TFPI; tissue factor pathway inhibitor isoform X1 [KO:K03909]
108389856  SERPINF2; alpha-2-antiplasmin isoform X1 [KO:K03983]
108391143  [KO:K03994]
108391144  [KO:K03994]
108391377  F8; coagulation factor VIII isoform X1 [KO:K03899]
108391597  VSIG4; LOW QUALITY PROTEIN: V-set and immunoglobulin domain-containing protein 4 [KO:K19822]
108392205  F13A1; coagulation factor XIII A chain isoform X1 [KO:K03917] [EC:2.3.2.13]
108392217  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
108393520  C7; complement component C7 isoform X1 [KO:K03996]
108393990  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
108393991  C2; complement C2 isoform X1 [KO:K01332] [EC:3.4.21.43]
108393997  C4A; complement C4-A [KO:K03989]
108394329  CLU; clusterin isoform X1 [KO:K17252]
108395527  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
108395779  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
108396404  F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
108396405  KLKB1; plasma kallikrein isoform X4 [KO:K01324] [EC:3.4.21.34]
108396895  F2RL3; proteinase-activated receptor 4 [KO:K04236]
108396921  THBD; thrombomodulin [KO:K03907]
108396937  VTN; vitronectin [KO:K06251]
108397532  CFI; complement factor I isoform X1 [KO:K01333] [EC:3.4.21.45]
108398548  [KO:K03984]
108398692  [KO:K03916]
108398703  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
108398704  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
108398764  [KO:K03913]
108398766  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
108398858  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
108399762  F3; tissue factor [KO:K03901]
108400695  ITGAM; integrin alpha-M [KO:K06461]
108401267  MASP1; mannan-binding lectin serine protease 1 isoform X3 [KO:K03992] [EC:3.4.21.-]
108401780  C3; complement C3 [KO:K03990]
108401888  MASP2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
108402121  [KO:K03991]
108402312  SERPING1; plasma protease C1 inhibitor [KO:K04001]
108402385  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
108402388  C8B; complement component C8 beta chain isoform X3 [KO:K03998]
108402421  PROS1; vitamin K-dependent protein S [KO:K03908]
108403113  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
108403283  C9; complement component C9 [KO:K04000]
108403296  F2; prothrombin isoform X1 [KO:K01313] [EC:3.4.21.5]
108403647  [KO:K06462]
108403861  C6; complement component C6 [KO:K03995]
108404459  VWF; von Willebrand factor isoform X1 [KO:K03900]
108405505  SERPINC1; antithrombin-III [KO:K03911]
108406293  FGB; fibrinogen beta chain isoform X1 [KO:K03904]
108406307  FGG; fibrinogen gamma chain [KO:K03905]
108406328  PROCR; endothelial protein C receptor [KO:K06557]
108406369  F12; coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
108406624  F13B; coagulation factor XIII B chain [KO:K03906]
108406838  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
108406900  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
108407308  C1QB; complement C1q subcomponent subunit B [KO:K03987]
108407588  CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
108408859  PLAUR; urokinase plasminogen activator surface receptor isoform X3 [KO:K03985]
108408979  SERPIND1; heparin cofactor 2 isoform X1 [KO:K03912]
Compound
C00290  Fibrin
C00306  Bradykinin
C00471  Plasmin
C00752  Thrombin
C01065  Coagulation factor Xa
C01715  Plasma kallikrein
C02002  C3a anaphylatoxin
C03259  Coagulation factor IXa
C03262  Coagulation factor XIa
C03378  Coagulation factor VIIa
C03379  Coagulation factor XIIa
C03780  Coagulation factor Va
C04001  Coagulation factor VIIIa
C16510  C5a anaphylatoxin
C22531  Complement component 2a
C22532  Complement component 2b
C22533  Complement component 3b
C22534  Complement component 3f
C22535  Complement iC3b
C22536  Complement component 3dg
C22537  Complement component 3c
C22538  Complement component 3d
C22539  Complement C3(H2O)
C22540  Complement factor Bb
C22541  Complement component 4a
C22542  Complement component 4b
C22543  Complement component 4c
C22544  Complement component 4d
C22545  Complement component 5b
C22546  Coagulation factor XIIIa
C22547  APC
Reference
  Authors
Bajic G, Degn SE, Thiel S, Andersen GR
  Title
Complement activation, regulation, and molecular basis for complement-related diseases.
  Journal
EMBO J 34:2735-57 (2015)
DOI:10.15252/embj.201591881
Reference
  Authors
Mathern DR, Heeger PS
  Title
Molecules Great and Small: The Complement System.
  Journal
Clin J Am Soc Nephrol 10:1636-50 (2015)
DOI:10.2215/CJN.06230614
Reference
  Authors
Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT
  Title
Complement System Part I - Molecular Mechanisms of Activation and Regulation.
  Journal
Front Immunol 6:262 (2015)
DOI:10.3389/fimmu.2015.00262
Reference
  Authors
Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD
  Title
Interactions between coagulation and complement--their role in inflammation.
  Journal
Semin Immunopathol 34:151-65 (2012)
DOI:10.1007/s00281-011-0280-x
Reference
  Authors
Kurosawa S, Stearns-Kurosawa DJ
  Title
Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
  Journal
J Intensive Care 2:65 (2014)
DOI:10.1186/s40560-014-0061-4
Reference
  Authors
Hillmeister P, Persson PB
  Title
The Kallikrein-Kinin system.
  Journal
Acta Physiol (Oxf) 206:215-9 (2012)
DOI:10.1111/apha.12007
Reference
  Authors
Rau JC, Beaulieu LM, Huntington JA, Church FC
  Title
Serpins in thrombosis, hemostasis and fibrinolysis.
  Journal
J Thromb Haemost 5 Suppl 1:102-15 (2007)
DOI:10.1111/j.1538-7836.2007.02516.x
Reference
  Authors
Crooks MG, Hart SP
  Title
Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
  Journal
Eur Respir Rev 24:392-9 (2015)
DOI:10.1183/16000617.00008414
Reference
  Authors
Christiaans SC, Wagener BM, Esmon CT, Pittet JF
  Title
Protein C and acute inflammation: a clinical and biological perspective.
  Journal
Am J Physiol Lung Cell Mol Physiol 305:L455-66 (2013)
DOI:10.1152/ajplung.00093.2013
Reference
  Authors
Alberelli MA, De Candia E
  Title
Functional role of protease activated receptors in vascular biology.
  Journal
Vascul Pharmacol 62:72-81 (2014)
DOI:10.1016/j.vph.2014.06.001
Reference
  Authors
Smith HW, Marshall CJ
  Title
Regulation of cell signalling by uPAR.
  Journal
Nat Rev Mol Cell Biol 11:23-36 (2010)
DOI:10.1038/nrm2821
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: pathophysiological mechanisms.
  Journal
J Immunol 190:3831-8 (2013)
DOI:10.4049/jimmunol.1203487
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: therapeutic interventions.
  Journal
J Immunol 190:3839-47 (2013)
DOI:10.4049/jimmunol.1203200
Reference
  Authors
Loof TG, Deicke C, Medina E
  Title
The role of coagulation/fibrinolysis during Streptococcus pyogenes infection.
  Journal
Front Cell Infect Microbiol 4:128 (2014)
DOI:10.3389/fcimb.2014.00128
Reference
  Authors
Jozsi M, Tortajada A, Uzonyi B, Goicoechea de Jorge E, Rodriguez de Cordoba S
  Title
Factor H-related proteins determine complement-activating surfaces.
  Journal
Trends Immunol 36:374-84 (2015)
DOI:10.1016/j.it.2015.04.008
Related
pathway
mjv04611  Platelet activation
mjv04662  B cell receptor signaling pathway
mjv04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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