PXD031620

PXD031620 is an original dataset announced via ProteomeXchange.

Title	Elucidation of the molecular changes underlying abnormal ER characteristic of osteosarcoma
Description	Pediatric osteosarcomas are characterized by an unusually dilated endoplasmic reticulum (ER). Our analysis of RNA expression data from 108 patient tumor samples and patient-derived xenografts (PDXs) revealed the overwhelming majority of these tumor have reduced expression of four COPII vesicle components that trigger aberrant accumulation of procollagen-I protein within the ER. CRISPR activation technology was used to increase expression of two of these, SAR1A and SEC24D, to physiologically normal levels. This was sufficient to resolve the dilated ER morphology and restore secretion of collagen-I, as well as enhance secretion of extracellular matrix (ECM) proteins. Our studies reveal the mechanism responsible for the dilated ER that is a hallmark characteristic of OS and identify a highly conserved molecular signature for this genetically unstable tumor.
HostingRepository	PRIDE
AnnounceDate	2022-05-20
AnnouncementXML	Submission_2022-05-20_06:25:02.284.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Yuxin Li
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	2022-02-11 06:37:27	ID requested
⏵ 1	2022-05-20 06:25:03	announced

Wood RK, Flory AR, Mann MJ, Talbot LJ, Hendershot LM, Secretory defects in pediatric osteosarcoma result from downregulation of selective COPII coatomer proteins. iScience, 25(4):104100(2022) [pubmed]

submitter keyword: Osteosarcoma, ER homeostasis, COPII vesicle components

Junmin Peng
contact affiliation	St. Jude Children's Research Hospital, Memphis, TN, USA
contact email	junmin.peng@stjude.org
lab head
Yuxin Li
contact affiliation	St. Jude Children's Research Hospital
contact email	yuxin.li@stjude.org
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/2022/05/PXD031620

PRIDE project URI

• PRIDE
  1. PXD031620
     1. Label: PRIDE project
     2. Name: Elucidation of the molecular changes underlying abnormal ER characteristic of osteosarcoma