Publication:
Visualizing the von Willebrand factor/glycoprotein Ib-IX axis with a platelet-type von Willebrand disease mutation

Simple item page
dc.contributor.authorKyei, Mark
dc.contributor.authorRussell, Susan
dc.contributor.authorLiu, Junling
dc.contributor.authorGartner, T Kent
dc.contributor.authorStorrie, Brian
dc.contributor.authorWare, Jerry
dc.contributor.authorGuerrero López, José Antonio
dc.contributor.departmentMedicina Interna
dc.contributor.otherFacultad de Medicina
dc.date.accessioned2026-01-28T09:34:26Z
dc.date.available2026-01-28T09:34:26Z
dc.date.copyright© 2009 by The American Society of Hematology
dc.date.issued2009-12-24
dc.description.abstractPlatelet-type von Willebrand disease (PT-VWD) is a bleeding disorder of the platelet glycoprotein Ib-IX/von Willebrand factor (VWF) axis caused by mutations in the glycoprotein Ib-IX receptor that lead to an increased affinity with VWF. In this report, platelets from a mouse expressing a mutation associated with PT-VWD have been visualized using state-of-the art image collection and processing. Confocal analysis revealed that VWF bound to the surface of single platelets and bridging micro-aggregates of platelets. Surface-bound VWF appears as a large, linear structure on the surface of 50% of the PT-VWD platelets. In vivo thrombus formation after chemical injury to the carotid artery revealed a severe impairment to occlusion as a consequence of the PT-VWD mutation. In vitro stimulation of PT-VWD platelets with adenosine diphosphate or thrombin demonstrates a significant block in their ability to bind fibrinogen. The impairment of in vivo thrombus formation and in vitro fibrinogen binding are more significant than might be expected from the observed platelet binding to VWF polymers over a small portion of the plasma membrane. Visualization of the receptor/ligand interaction and characterization of a severe antithrombotic phenotype provide a new understanding on the molecular basis of bleeding associated with the PT-VWD phenotype.
dc.formatapplication/pdf
dc.format.extent7
dc.identifier.citationBlood (2009) 114 (27): 5541–5546
dc.identifier.doihttps://doi.org/10.1182/blood-2009-03-210823
dc.identifier.eissn1528-0020
dc.identifier.issn0006-4971
dc.identifier.urihttp://hdl.handle.net/10201/195809
dc.languageeng
dc.publisherAmerican Society of Hematology (ASH Publications)
dc.relationThis work was supported by funds from the American National Heart, Lung, and Blood Institute (HL50545; J.W.). J.A.G. is supported by a Sara Borrell postdoctoral contract from Instituto de Salud Carlos III
dc.relation.publisherversionhttps://ashpublications.org/blood/article/114/27/5541/26579/Visualizing-the-von-Willebrand-factor-glycoprotein
dc.rightsAttribution-NonCommercial-NoDerivates 4.0 International
dc.rights.accessRightsinfo:eu-repo/semantics/openAccess
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subject.odsObjetivo 3: Salud
dc.titleVisualizing the von Willebrand factor/glycoprotein Ib-IX axis with a platelet-type von Willebrand disease mutation
dc.typeinfo:eu-repo/semantics/article
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dspace.entity.typePublication
relation.isAuthorOfPublication099fb9a8-9bae-43fb-be04-b8ea37502882
relation.isAuthorOfPublication.latestForDiscovery099fb9a8-9bae-43fb-be04-b8ea37502882
Files
Original bundle
Now showing 1 - 1 of 1
Thumbnail Image
Name:
2009_Blood.pdf
Size:
678.98 KB
Format:
Adobe Portable Document Format
Description:
Download
License bundle
Now showing 1 - 1 of 1
Thumbnail Image
Name:
license.txt
Size:
1.37 KB
Format:
Item-specific license agreed upon to submission
Description:
Download
Collections
Artículos