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PXD003912

PXD003912 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleNew alterations of the platelet proteome in type I Glanzmann thrombasthenia
DescriptionThe project contains raw and result files of a proteomics analysis of two patients with type 1 Glanzmann thrombasthenia (Patient1 and 2) caused by homozygous ITGA2b delG mutations and from family (heterozygous: Father1, Mother1, Sister1, healthy: Sister2) and unrelated controls. A label-free analysis has been conducted in duplicates using Progenesis (NonLinear Dynamics) demonstrating that less than 5% of glycoprotein IIb and 5-7% fibrinogen is expressed compared to healthy controls. Heterozygous familiy members from one of the controls expressed 50-60% of GPIIb sufficient for normal GPIIb/IIIa-dependant platelet function. In a subsequent targeted LC-MS/MS analysis we quantified 14 protein identified as potentially regulated in preliminary iTRAQ experiments. We observed diminished expession of factor XIIIB chain (P05160), plasminogen (P00747) and carboxypeptidase 2B (Q96IY4) in thrombasthenic platelets as well as a 2.5-fold increased expression of FcgRIIA (P12318/P31995) and laminin alpha4 (Q16363).
HostingRepositoryPRIDE
AnnounceDate2024-10-22
AnnouncementXMLSubmission_2024-10-22_04:05:11.146.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterStefan Loroch
SpeciesList scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationListiodoacetamide derivatized residue
InstrumentLTQ Orbitrap Velos; Q Exactive
Dataset History
RevisionDatetimeStatusChangeLog Entry
02016-04-06 00:05:51ID requested
12019-01-14 07:53:51announced
22024-10-22 04:05:12announced2024-10-22: Updated project metadata.
Publication List
10.1160/th16-07-0515;
Loroch S, Trabold K, Gambaryan S, Rei, ß C, Schwierczek K, Fleming I, Sickmann A, Behnisch W, Zieger B, Zahedi RP, Walter U, Jurk K, Alterations of the platelet proteome in type I Glanzmann thrombasthenia caused by different homozygous delG frameshift mutations in ITGA2B. Thromb Haemost, 117(3):556-569(2017) [pubmed]
Keyword List
curator keyword: Biomedical
submitter keyword: Glanzmann thrombasthenia, LC-MS,Platelets, Proteomics
Contact List
Albert Sickmann
contact affiliationLeibniz-Institut für Analytische Wissenschaften - ISAS - e.V. Otto-Hahn-Strasse 6b 44227 Dortmund Germany
contact emailsickmann@isas.de
lab head
Stefan Loroch
contact affiliationLeibniz-Institut für Analytische Wissenschaften, Otto-Hahn-Straße 6b, 44227 Dortmund
contact emailstefan.loroch@isas.de
dataset submitter
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Dataset FTP location
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PRIDE project URI
Repository Record List
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