<<< Full experiment listing

PXD037759-1

PXD037759 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleIRF1 mass-spectrometry primary fibroblasts non-stimulated or stimulated with IFNg for 24h
DescriptionInborn errors of human IFN-γ immunity underlie mycobacterial diseases, whereas inborn errors of IFN-a/b immunity underlie viral diseases. Both types of IFNs induce the transcription factor IRF1. We describe two unrelated children with inherited complete IRF1 deficiency and early-onset, multiple, life-threatening diseases caused by weakly virulent mycobacteria. These children have no history of severe viral disease, despite exposure to many viruses, including SARS-CoV-2, which is life-threatening in individuals with impaired IFN-a/b immunity. The IRF1-dependent cellular responses to IFN-γ are, both quantitatively and qualitatively, much greater than those to IFN-a/b in vitro. Monocyte- and iPSC-derived macrophages from the two patients show no upregulation of at least 20% of the target genes normally induced by IFN-γ. By contrast, cell-intrinsic IFN-a/b immunity to diverse viruses, including SARS-CoV-2, is intact. Human IRF1 is, thus, largely redundant for antiviral IFN-a/b immunity. By contrast, human IRF1 is essential for IFN-γ immunity to mycobacteria in myeloid cells.
HostingRepositoryPRIDE
AnnounceDate2023-01-19
AnnouncementXMLSubmission_2023-01-19_08:42:23.069.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterSorenHeissel
SpeciesList scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationListNo PTMs are included in the dataset
InstrumentOrbitrap Fusion Lumos
Dataset History
RevisionDatetimeStatusChangeLog Entry
02022-10-27 02:38:25ID requested
12023-01-19 08:42:23announced
22023-11-14 08:59:45announced2023-11-14: Updated project metadata.
Publication List
Keyword List
submitter keyword: fibroblasts, IFNg, LC-MS
Contact List
Jean LaurentCasanova
contact affiliationSt. Giles Laboratory of Human Genetics of Infectious Diseases, The Rockefeller University, New York, New York, USA.
contact emailcasanova@rockefeller.edu
lab head
SorenHeissel
contact affiliationResearch Support Specialist
contact emailsheissel@rockefeller.edu
dataset submitter
Full Dataset Link List
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/2023/01/PXD037759
PRIDE project URI
Repository Record List
[ + ]