PXD023933

PXD023933 is an original dataset announced via ProteomeXchange.

Title	Comparative proteome research in a zebrafish model for Vanishing White Matter disease
Description	Vanishing white matter (VWM) disease is a genetic leukodystrophy leading to severe neurological disease and early death. VWM is caused by bi-allelic mutations in any of the five genes encoding the subunits of the eukaryotic translation factor 2B (EIF2B). Although previous studies are being attempted to investigate the molecular mechanism of VWN by constructing variant models for each subunit of EIF2B that causes VWM disease. The underlying molecular mechanism of how mutations (MT) in EIF2B3 result in VWM is unknown. Based on our recently report, we constructed an eif2b3 knockout (eif2b3-/-) zebrafish (Danio rerio) model and performed quantitative proteomic analysis between the wide type (WT) and eif2b3-/- models to identify 25 differentially expressed proteins (DEPs). A total of 4 proteins were significantly up-regulated, and 21 proteins were significantly down-regulated in eif2b3-/- larvae compared to WT, respectively. Representatively, Lon protease and neutral amino acid transporter SLC1A4 were significantly increased in truncated eif2b3 zebrafish, and crystallin proteins were also significantly decreased. In conclusion, this study proposed the candidate proteins as a key regulator related to the progression of VWN disease through quantitative proteomic analysis in the first variant model of VWN disease.
HostingRepository	PRIDE
AnnounceDate	2021-09-10
AnnouncementXML	Submission_2021-09-09_23:49:30.175.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Doeun Kim
SpeciesList	scientific name: Danio rerio (Zebrafish) (Brachydanio rerio); NCBI TaxID: 7955;
ModificationList	No PTMs are included in the dataset
Instrument	LTQ Orbitrap Velos

Revision	Datetime	Status	ChangeLog Entry
0	2021-02-02 01:42:46	ID requested
⏵ 1	2021-09-09 23:49:30	announced

Kim D, Lee YR, Choi TI, Kim SH, Kang HC, Kim CH, Lee S, Comparative Proteome Research in a Zebrafish Model for Vanishing White Matter Disease. Int J Mol Sci, 22(5):(2021) [pubmed]

submitter keyword: eif2b3, Vanishing White Matter Disease, comparative proteomics, SLC1A4

Sangkyu Lee
contact affiliation	College of Pharmacy, Research Institute of Pharmaceutical Sciences, Kyungpook National University, Republic of Korea
contact email	sangkyu@knu.ac.kr
lab head
Doeun Kim
contact affiliation	College of Pharmacy and Research Institute of Pharmaceutical Sciences, Kyungpook National University, Daegu 41566, Republic of Korea
contact email	kdkdl1230@gmail.com
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/2021/09/PXD023933

PRIDE project URI

• PRIDE
  1. PXD023933
     1. Label: PRIDE project
     2. Name: Comparative proteome research in a zebrafish model for Vanishing White Matter disease