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Regulation of clotting cascade

Summary

Organism

Homo sapiens (human)

Reactome

R-HSA-9769739

PubChem

R-HSA-9769739

Description

The coagulation pathway is tightly regulated by numerous mechanisms to maintain hemostatic balance and ensure effective clot formation. Dysregulation of any component can lead to bleeding or thrombotic disorders. This Reactome module describes key endogenous regulators of the clotting pathway, including:
Tissue factor pathway inhibitor (TFPI): Forms a quaternary complex with FXa, FVIIa and TF, effectively inactivating FXa and FVIIa to restrict coagulation during the initiation phase (Broze GJ et al., 1990; Maroney SA et al., 2013).
Protein C: Activated by the thrombin–thrombomodulin complex on intact endothelial cells. Together with its cofactor, protein S, it inhibits FVa and FVIIIa activities, contributing to anticoagulation (Stavenuiter F et al., 2013).
Serine endopeptidase inhibitors, including SERPINC1 (antithrombin III), SERPINA5, SERPINA10 and SERPIND1: Inactivate serine proteases such as thrombin and FXa by irreversibly binding to their serine activation sites (Mushunje A et al., 2003; Boulaftali Y et al., 2010; Huang X et al., 2010).
A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13): A metalloprotease, that regulates thrombus formation by cleaving VWF multimers on the platelet surface or in the circulation in the presence of factor VIII (Cao W et al., 2008; DeYoung V et al., 2022;

This Reactome module also highlights selected drugs that target clotting factors or their regulators to promote effective clot formation (Petersen LC 2012; Nutescu EA et al., 2016).

Click on a node on the pathway to see its details. Glycoproteins are marked with a glycoprotein icon in their name.

SPV: a JavaScript Signaling Pathway Visualizer, Bioinformatics, 2018

Displaying entries **1 - 10** of 45 in total

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UniProt ID	Protein Name	Gene Symbol	Pathway Viewer
O00468	Agrin	AGRIN AGRN	view
O75056	Syndecan-3	KIAA0468 SDC3	view
O75487	Glypican-4	GPC4 UNQ474/PRO937	view
P00451	Coagulation factor VIII	F8 F8C	view
P00734	Prothrombin	F2	view
P00740	Coagulation factor IX	F9	view
P00742	Coagulation factor X	F10	view
P01008	Antithrombin-III	AT3 PRO0309 SERPINC1	view
P03951	Coagulation factor XI	F11	view
P04070	Vitamin K-dependent protein C	PROC	view

Displaying **all 3** entries
GlyCosmos Lectin	UniProt ID	Lectin Name	Pathway Viewer
GL_005327	O00468	Agrin	view
GL_005334	P98160	Basement membrane-specific heparan sulfate proteoglycan core protein	view
GL_005529	P07204	Thrombomodulin	view

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