PXD056734

PXD056734 is an original dataset announced via ProteomeXchange.

Title	The proteomic landscape of platelets in Glanzmann thrombasthenia
Description	Background: Glanzmann thrombasthenia (GT) is a rare, inherited platelet function disorder caused by mutations in the integrins of the fibrinogen receptor αIIbβ3. The deficiency can be quantitative (type I/II) or qualitative (type III). This causes lack of platelet aggregation and consequently these patients suffer from a moderate to severe bleeding tendency. Besides the absence or functional alteration of the integrins αIIb and β3, little is known about the proteomic landscape of platelets from people with GT. Objectives: We aim to evaluate the proteomic landscape of platelets in GT. Patient/Methods: Thirteen patients with GT from the Thrombocytopathy in the Netherlands (TiN) study and healthy controls were included in this study. Whole blood flow cytometry analysis was performed to quantify αIIbβ3 expression. GT was genetically confirmed. Platelets were isolated and prepared for liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS). Results: Flow cytometry and LC-MS/MS based αIIb expression correlated well and resulted in 11 type I GT and 2 type III GT patients. Analysis of the LC-MS/MS data revealed similar platelet proteome in GT vs healthy platelets, with downregulation of a few platelet specific proteins that are associated with α-granules. Upregulated proteins in GT were almost all genuine plasma proteins rather than platelet specific proteins. Conclusions: The proteomic landscape of GT platelets is overall similar as in healthy platelets, with downregulation of some platelet specific α-granule proteins.
HostingRepository	PRIDE
AnnounceDate	2025-05-12
AnnouncementXML	Submission_2025-05-12_04:58:47.066.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Tatiana Shamorkina
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2024-10-11 09:15:32	ID requested
⏵ 1	2025-05-12 04:58:47	announced

10.1016/J.JTHA.2025.04.004;

submitter keyword: Glanzmann

platelets

proteomics of platelets

Albert JR Heck
contact affiliation	Biomolecular Mass Spectrometry and Proteomics, Bijvoet Center for Biomolecular Research and Utrecht Institute for Pharmaceutical Sciences, Utrecht University, Utrecht, The Netherlands
contact email	a.j.r.heck@uu.nl
lab head
Tatiana Shamorkina
contact affiliation	Utrecht University
contact email	t.m.shamorkina@uu.nl
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2025/05/PXD056734

PRIDE project URI

• PRIDE
  1. PXD056734
     1. Label: PRIDE project
     2. Name: The proteomic landscape of platelets in Glanzmann thrombasthenia