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GP1b-IX-V activation signalling

Summary

Organism

Homo sapiens (human)

Reactome

R-HSA-430116

PubChem

R-HSA-430116

Description

The platelet GPIb complex (GP1b-IX-V) together with GPVI are primarily responsible for regulating the initial adhesion of platelets to the damaged blood vessel and platelet activation. The importance of GPIb is demonstrated by the bleeding problems in patients with Bernard-Soulier syndrome where this receptor is either absent or defective. GP1b-IX-V binds von Willebrand Factor (vWF) to resting platelets, particularly under conditions of high shear stress. This transient interaction is the first stage of the vascular repair process. Activation of GP1b-IX-V on exposure of the fibrous matrix following atherosclerotic plaque rupture, or in occluded arteries, is a major contributory factor leading to thrombus formation leading to heart attack or stroke. GpIb also binds thrombin (Yamamoto et al. 1986), at a site distinct from the site of vWF binding, acting as a docking site for thrombin which then activates Proteinase Activated Receptors leading to enhanced platelet activation (Dormann et al. 2000).

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 **all 10** entries
UniProt ID	Protein Name	Gene Symbol	Pathway Viewer
P02452	Collagen alpha-1(I) chain	COL1A1	view
P04049	RAF proto-oncogene serine/threonine-protein kinase	RAF RAF1	view
P04275	von Willebrand factor	F8VWF VWF	view
P07359	Platelet glycoprotein Ib alpha chain	GP1BA	view
P08123	Collagen alpha-2(I) chain	COL1A2	view
P13224	Platelet glycoprotein Ib beta chain	GP1BB	view
P14770	Platelet glycoprotein IX	GP9	view
P21333	Filamin-A	FLN FLN1 FLNA	view
P40197	Platelet glycoprotein V	GP5	view
P63104	14-3-3 protein zeta/delta	YWHAZ	view

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