PXD042180-1

PXD042180 is an original dataset announced via ProteomeXchange.

Dataset Summary

Title	NBEAL2 interactome: comparison between healthy donors and grey platelet syndrome (NBEAL2 deficient) patients activated T cells samples.
Description	Loss of NBEAL2 function leads to grey platelet syndrome (GPS), a bleeding disorder characterized by macro-thrombocytopenia and α-granule-deficient platelets. A proportion of patients with GPS develop autoimmunity through an unknown mechanism, which might be related to the proteins NBEAL2 interacts with, specifically in immune cells. Here we show a comprehensive interactome of NBEAL2 in primary T cells, based on mass spectrometry identification of altogether 76 protein association partners. These include LRBA, a member of the same BEACH domain family as NBEAL2, recessive mutations of which cause autoimmunity and lymphocytic infiltration through defective CTLA-4 trafficking
HostingRepository	jPOST
AnnounceDate	2023-05-12
AnnouncementXML	Submission_2023-05-12_06:57:17.129.xml
DigitalObjectIdentifier
ReviewLevel	Non peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Armelle BUZY
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	S-carboxamidomethyl-L-cysteine; alpha-amino acetylated residue; L-methionine sulfoxide; 2-pyrrolidone-5-carboxylic acid (Gln); CAMthiopropanoyl
Instrument	instrument

Dataset History

Revision	Datetime	Status	ChangeLog Entry
0	2023-05-12 06:21:09	ID requested
⏵ 1	2023-05-12 06:57:17	announced

Publication List

Dataset with its publication pending

Dataset with its publication pending

Keyword List

submitter keyword: NBEAL2, LRBA, CTLA-4, Autoimmunity, gray platelet syndrome, BEACH domain, BEACH-domain-containing protein, interactome, immune checkpoint

submitter keyword: NBEAL2, LRBA, CTLA-4, Autoimmunity, gray platelet syndrome, BEACH domain, BEACH-domain-containing protein, interactome, immune checkpoint

Contact List

Dr. Frédéric Rieux-Laucat
lab head
Armelle BUZY
contact affiliation	SANOFI
dataset submitter

Full Dataset Link List

jPOST dataset URI
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jPOST dataset URI

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.jpostdb.org/JPST002157/