Complement and Coagulation Cascades (Homo sapiens)

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Muscle contraction,Chemotaxis,Phagocyte recruitmentInflammation, etc.Vascular injuryClassical PathwayC3b/BbDegredationFibrinolytic systemZymosan,insulin, etc.membrane attack complexCell lysisMediatorsfibrin degredationproductsC4aC5aLectin PathwayC3b/4b, C3b, C3dCoagulation CascadeC3ConvertaseComplement CascadeAntibody/antigencomplexExtrinsic pathwayCross-linkedfibrin polymerKinin kallikrein systemCell adhesion,migration,profileration, etcAlternative PathwayClotC4b/2aCell adhesion,migration,profileration, etcC3aPlatelet, monocyte,lymphocyte,endothelial cells,smooth muscle cellsactivation, etcC3bThrombinCollagen,basement membrane,activated platelets, etc.PrM/CapCFIF8ADNPrM/CapF9C1QASERPINF2TFPIPROS1F3C1QB109821PLGF12C1RF5C6C7MBL1PLATDaf1C1SSERPINC1C3C9BDKRB1F7MASP2F10KNG1MCPCR2FGBCLUDaf2Fibrin monomerC3AR1VWFC2SERPINE1F13BTHBDCRRYMASP1C5R1CR1CD59ACFHC1QGPLAUBradykininH2-BFHcF2F2RPROCC8GPLAURSERPINA5SERPING1APOA2A2MCPB2KLKB1C4SERPINA1SERPIND1


Blood coagulation is a series of coordinated and calcium-dependent proenzyme-to-serine protease conversions likely to be localized on the surfaces of activated cells in vivo. It culminates in the formation of thrombin, the enzyme responsible for the conversion of soluble fibrinogen to the insoluble fibrin clot.

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. Complement is a system of plasma proteins that is activated by the presence of 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, intern, generates the effector molecules of complement. The three main consequences of complement activation are the opsonization of pathogens, the recruitment of inflammatory and immunocompetent cells, and the direct killing of pathogens.

Source: KEGG (


GenMAPP notes
GenMAPP remarks 
Adapted from KEGG

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Ontology Terms



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106553view00:19, 6 September 2019Khanspersfixed interactions
106365view23:04, 22 August 2019KhanspersModified description
105558view06:16, 9 August 2019KhanspersModified description
95109view14:37, 16 November 2017DeSlFixed nested groups.
90196view21:15, 21 October 2016Khanspersfixed unconnected
90009view23:21, 7 October 2016AlexanderPicoRemoved dengue-2 interactions; moved to new pathway, WP3896
90005view17:47, 7 October 2016MaintBotRemoved old genmapp comments
85997view14:44, 23 June 2016FehrhartCorrected another typo
85996view12:57, 23 June 2016LindarieswijkCorrected some spelling errors
79680view12:52, 4 April 2015EgonwFixed non-numeric Entrez identifiers: looked up numeric identifier or if not found in human, removed it.
79567view18:40, 25 March 2015Zariadded ID for Daf1,H2-BF,CFH and CLU genes
67786view15:14, 26 June 2013DdiglesOntology Term : 'complement system pathway' added !
63170view20:26, 8 May 2013MaintBotUpdating gpml version
59169view18:34, 22 February 2013MaintBotUpdated Ensembl and UniProt data source
45058view19:54, 6 October 2011AlexanderPicoadded db ref
41101view23:22, 1 March 2011MaintBotRemoved redundant pathway information and comments
38701view19:26, 23 September 2010AlexanderPico
36243view19:16, 11 March 2010LslabimD2 coag comp
36242view19:12, 11 March 2010LslabimPeriodical save, work in progress
36241view18:41, 11 March 2010LslabimD2 effect coag comp
36240view18:32, 11 March 2010LslabimPeriodical save, work in progress
36239view18:21, 11 March 2010LslabimPeriodical save, work in progress
34809view00:23, 21 January 2010KhanspersChanged comment
34808view00:22, 21 January 2010KhanspersModified description
34807view00:20, 21 January 2010KhanspersModified description
34467view18:30, 10 December 2009MaintBotAutomatic update of empty xrefs
32465view17:28, 15 August 2009MaintBotFixed text labels
32149view14:01, 14 August 2009MaintBotFixed group labels
22582view13:34, 14 November 2008Thomaschanged name (was duplicate with WP545)
21854view11:32, 14 November 2008MaintBot[[Pathway:Homo sapiens:Complement and Coagulation Cascades KEGG]] moved to [[Pathway:WP558]]: Moved to stable identifier
14616view07:15, 23 May 2008Thomasadded groups and linked lines
14612view07:09, 23 May 2008ThomasPeriodical save, work in progress
14608view06:49, 23 May 2008ThomasPeriodical save, work in progress
13052view11:11, 17 May 2008MaintBotautomated metabolite conversion
12930view10:41, 17 May 2008MaintBotSticky edges patch by Sjoerd
7972view13:46, 7 January 2008MaintBot[[Pathway:Human:Complement and Coagulation Cascades KEGG]] moved to [[Pathway:Homo sapiens:Complement and Coagulation Cascades KEGG]]: Renaming species
7610view16:04, 18 December 2007MaintBotfixed category names
7467view12:42, 4 November 2007MaintBotAdded categories to GPML
6305view22:17, 22 May 2007169.230.77.174gpml file for [[Human:Complement_and_Coagulation_Cascades_KEGG]]

External references


View all...
NameTypeDatabase referenceComment
109821GeneProduct109821 (Entrez Gene)
A2MGeneProduct2 (Entrez Gene)
ADNGeneProductADN (Entrez Gene)
BDKRB1GeneProduct623 (Entrez Gene)
BradykininMetabolite58-82-2 (CAS)
C1QAGeneProduct712 (Entrez Gene)
C1QBGeneProduct713 (Entrez Gene)
C1QGGeneProduct714 (Entrez Gene)
C1RGeneProduct715 (Entrez Gene)
C1SGeneProduct716 (Entrez Gene)
C2GeneProduct717 (Entrez Gene)
C3GeneProduct718 (Entrez Gene)
C3AR1GeneProduct719 (Entrez Gene)
C4GeneProductC4 (Entrez Gene)
C5R1GeneProduct728 (Entrez Gene)
C6GeneProduct729 (Entrez Gene)
C7GeneProduct730 (Entrez Gene) MGI:88235 LL:109828
C8GGeneProduct733 (Entrez Gene)
C9GeneProduct735 (Entrez Gene)
CD59AGeneProductCD59A (Entrez Gene)
CFHGeneProductCFH (Entrez Gene)
CFIGeneProductCFI (Entrez Gene)
CLUGeneProductCLU (Entrez Gene)
  • CLU
  • Type your comment here
CPB2GeneProduct1361 (Entrez Gene)
CR1GeneProduct1378 (Entrez Gene)
CR2GeneProduct1380 (Entrez Gene)
CRRYGeneProductCRRY (Entrez Gene)
Daf1GeneProductDaf1 (Entrez Gene)
Daf2GeneProductDaf2 (Entrez Gene)
F10GeneProduct2159 (Entrez Gene)
F12GeneProduct2161 (Entrez Gene)
F13BGeneProduct2165 (Entrez Gene)
F2GeneProduct2147 (Entrez Gene)
F2RGeneProduct2149 (Entrez Gene)
F3GeneProduct2152 (Entrez Gene)
F5GeneProduct2153 (Entrez Gene)
F7GeneProduct2155 (Entrez Gene)
F8GeneProduct2157 (Entrez Gene)
F9GeneProduct2158 (Entrez Gene)
FGBGeneProduct2244 (Entrez Gene)
Fibrin monomerMetabolite9001-31-4 (CAS)
H2-BFGeneProductH2-BF (Entrez Gene)
HcGeneProductHc (Entrez Gene)
KLKB1GeneProduct3818 (Entrez Gene)
KNG1GeneProduct3827 (Entrez Gene)
MASP1GeneProduct5648 (Entrez Gene)
MASP2GeneProduct10747 (Entrez Gene)
MBL1GeneProductMBL1 (Entrez Gene)
MCPGeneProduct4179 (Entrez Gene)
PLATGeneProduct5327 (Entrez Gene)
PLAUGeneProduct5328 (Entrez Gene)
PLAURGeneProduct5329 (Entrez Gene)
PLGGeneProduct5340 (Entrez Gene)
PROCGeneProduct5624 (Entrez Gene)
PROS1GeneProduct5627 (Entrez Gene)
SERPINA1GeneProduct5265 (Entrez Gene)
SERPINA5GeneProduct5104 (Entrez Gene)
SERPINC1GeneProduct462 (Entrez Gene)
SERPIND1GeneProduct3053 (Entrez Gene)
SERPINE1GeneProduct5054 (Entrez Gene)
SERPINF2GeneProduct5345 (Entrez Gene)
SERPING1GeneProduct710 (Entrez Gene)
TFPIGeneProduct7035 (Entrez Gene)
THBDGeneProduct7056 (Entrez Gene)
VWFGeneProduct7450 (Entrez Gene)

Annotated Interactions

No annotated interactions
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