PXD001197

PXD001197 is an original dataset announced via ProteomeXchange.

Title	SIL1-depleted HEK293 cells - Cellular Signature of SIL1 Depletion: Disease Pathogenesis due to Alterations in Protein Composition Beyond the ER Machinery
Description	Marinesco-Sjögren syndrome (MSS) is a neurodegenerative disorder caused by autosomal recessive SIL1 mutations. SIL1 acts as a nucleotide exchange factor for the endoplasmic reticulum (ER) resident chaperone BiP. As BiP controls many ER-related processes, it is likely to contribute to MSS pathology. Owing to the absence of appropriate in vitro models, the precise pathophysiological mechanisms leading to neurodegeneration in MSS are still elusive. Here, we demonstrate for the first time that SIL1-depleted HEK293 cells can be used to reveal these mechanisms using ultra-structural, cell biological, and biochemical approaches.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:07:00.591.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	René Zahedi
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	LTQ Orbitrap Elite

Revision	Datetime	Status	ChangeLog Entry
0	2014-08-05 00:22:42	ID requested
1	2017-02-28 05:17:04	announced
⏵ 2	2024-10-22 04:07:03	announced	2024-10-22: Updated project metadata.

10.1007/s12035-015-9456-z;

Roos A, Kollipara L, Buchkremer S, Labisch T, Brauers E, Gatz C, Lentz C, Gerardo-Nava J, Weis J, Zahedi RP, Cellular Signature of SIL1 Depletion: Disease Pathogenesis due to Alterations in Protein Composition Beyond the ER Machinery. Mol Neurobiol, 53(8):5527-41(2016) [pubmed]

curator keyword: Biomedical

submitter keyword: HEK293, SIL1, proteome

René Peiman Zahedi
contact affiliation	Leibniz – Institut für Analytische Wissenschaften - ISAS - e.V.
contact email	rene.zahedi@isas.de
lab head
René Zahedi
contact affiliation	Segal Cancer Proteomics Centre
contact email	Rene.Zahedi@ladydavis.ca
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/2017/02/PXD001197

PRIDE project URI

• PRIDE
  1. PXD001197
     1. Label: PRIDE project
     2. Name: SIL1-depleted HEK293 cells - Cellular Signature of SIL1 Depletion: Disease Pathogenesis due to Alterations in Protein Composition Beyond the ER Machinery