PXD002742

PXD002742 is an original dataset announced via ProteomeXchange.

Title	Quantitative Proteomics Reveals Metabolic Reprogramming during ETHE1 Deficiency, Part 2
Description	Deficiency of mitochondrial sulfur dioxygenase (ETHE1) causes the severe metabolic disorder ethylmalonic encephalopathy, which is characterized by early-onset encephalopathy and defective cytochrome C oxidase because of hydrogen sulfide accumulation. Although the severe systemic consequences of the disease are becoming clear, the molecular effects are not well defined. Therefore, for further elucidating the effects of ETHE1-deficiency, we performed a large scale quantitative proteomics study on liver tissue from ETHE1-deficient mice. Our results demonstrated a clear link between ETHE1-deficiency and redox active proteins, as reflected by down-regulation of several proteins related to oxidation-reduction, such as different dehydrogenases and cytochrome P450 (CYP450) members. Furthermore, the protein data indicated impact of the ETHE1-deficiency on metabolic reprogramming through up-regulation of glycolytic enzymes and by altering several heterogeneous ribonucleoproteins (hnRNPs), indicating novel link between ETHE1 and gene expression. We also found increase in total protein acetylation level, pointing out the link between ETHE1 and acetylation, which is likely controlled by both redox state and cellular metabolites. These findings are relevant for understanding the complexity of the disease and may shed light on important functions influenced by ETHE1 deficiency and by the concomitant increase in the gaseous mediator hydrogen sulfide.
HostingRepository	PRIDE
AnnounceDate	2016-04-11
AnnouncementXML	Submission_2016-04-11_01:01:09.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD002742
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Supported dataset by repository
PrimarySubmitter	Johan Palmfeldt
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	No PTMs are included in the dataset
Instrument	LTQ Orbitrap

Revision	Datetime	Status	ChangeLog Entry
0	2015-08-17 07:13:54	ID requested
⏵ 1	2016-04-11 01:01:10	announced

Sahebekhtiari N, Thomsen MM, Sloth JJ, Stenbroen V, Zeviani M, Gregersen N, Viscomi C, Palmfeldt J, Quantitative proteomics suggests metabolic reprogramming during ETHE1 deficiency. Proteomics, 16(7):1166-76(2016) [pubmed]

curator keyword: Biological, Biomedical

submitter keyword: Liver, LC-MS/MS, iTRAQ, mouse, ETHE1, persulfide dioxygenase, acetylation, metabolism, sulfide

Johan Palmfeldt
contact affiliation	Research Unit for Molecular Medicine, Department of Clinical Medicine, Aarhus University and Aarhus University Hospital, Aarhus, Denmark
contact email	johan.palmfeldt@clin.au.dk
lab head
Johan Palmfeldt
contact affiliation	Aarhus University Hospital
contact email	johan.palmfeldt@clin.au.dk
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/2016/04/PXD002742

PRIDE project URI

• PRIDE
  1. PXD002742
     1. Label: PRIDE project
     2. Name: Quantitative Proteomics Reveals Metabolic Reprogramming during ETHE1 Deficiency, Part 2