PXD017946

PXD017946 is an original dataset announced via ProteomeXchange.

Title	11q deletion or ALK activity curbs DLG2 expression to maintain an undifferentiated state in neuroblastoma.
Description	High-risk 11q deleted neuroblastomas (NBs) typically display an undifferentiated/poorly differentiated morphology. NB is thought to develop from Schwann cell precursors (SCPs) and un-differentiated neural crest derived cells (NCC). It is therefore vital to understand the mechanisms involved in the block of differentiation. We showed that an important and novel role for oncogenic ALK-ERK1/2-SP1 signaling may be the maintenance of an undifferentiated state of transformed NC-derived progenitors that is achieved by repression of DLG2, a tumor suppressor in NB. DLG2 is expressed in the ‘bridge signature’ that represents the transcriptional transition state when neural crest cells or Schwann Cell Precursors (SCP) become chromaffin cells of the adrenal gland. The importance of SP1 and DLG2 in this process is highlighted by our findings that restoring DLG2 expression spontaneously drives NB differentiation. Further, genetic analysis of high-risk 11q deletion NB patient identified genetic lesions in the DLG2 gene, Our data also suggest that further exploration of other ‘bridge genes’ may help to better understand the mechanisms underlying the differentiation of NC-derived progenitors and their contribution to NB.
HostingRepository	PRIDE
AnnounceDate	2020-11-25
AnnouncementXML	Submission_2020-11-24_23:04:48.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Evelin Berger
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	methylthiolated residue; monohydroxylated residue
Instrument	Orbitrap Fusion

Revision	Datetime	Status	ChangeLog Entry
0	2020-03-09 06:25:10	ID requested
1	2020-11-24 22:53:47	announced
⏵ 2	2020-11-24 23:04:49	announced	2020-11-25: Updated publication reference for PubMed record(s): 32966799.

Siaw JT, Javanmardi N, Van den Eynden J, Lind DE, Fransson S, Martinez-Monleon A, Djos A, Sj, ö, berg RM, Ö, stensson M, Car, é, n H, Tr, ø, en G, Beiske K, Berbegall AP, Noguera R, Lai WY, Kogner P, Palmer RH, Hallberg B, Martinsson T, 11q Deletion or ALK Activity Curbs DLG2 Expression to Maintain an Undifferentiated State in Neuroblastoma. Cell Rep, 32(12):108171(2020) [pubmed]

submitter keyword: ALK, neuroblastoma, DLG2, tumor suppressor, Retinoic acid, ERK, TRK, NGF, SNP, genomic profiles, whole genome sequencing

Carina Sihlbom
contact affiliation	Proteomics Core Facility, University of Gothenburg, Sweden
contact email	carina.sihlbom@gu.se
lab head
Evelin Berger
contact affiliation	University of Gothenburg
contact email	evelin.berger@gu.se
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/2020/11/PXD017946

PRIDE project URI

• PRIDE
  1. PXD017946
     1. Label: PRIDE project
     2. Name: 11q deletion or ALK activity curbs DLG2 expression to maintain an undifferentiated state in neuroblastoma.