PXD009880

PXD009880 is an original dataset announced via ProteomeXchange.

Dataset Summary

Title	LC-MS/MS proteomics of ETHE1 deficient patient fibroblasts_Study-1
Description	The mitochondrial enzyme ETHE1 is a persulfide dioxygenase essential for cellular sulfide detoxification, and its deficiency causes the severe and complex inherited metabolic disorder ethylmalonic encephalopathy (EE). In spite of well-described clinical symptoms of the disease, detailed cellular and molecular characterization is still ambiguous. TMT-based large-scale proteomics was performed to broadly elucidate cellular consequences of the ETHE1 deficiency in the patient fibroblasts.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:12:20.764.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Johan Palmfeldt
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive

Dataset History

Revision	Datetime	Status	ChangeLog Entry
0	2018-05-24 03:35:05	ID requested
1	2018-11-07 01:05:31	announced
⏵ 2	2024-10-22 04:12:21	announced	2024-10-22: Updated project metadata.

Publication List

Sahebekhtiari N, Fernandez-Guerra P, Nochi Z, Carlsen J, Bross P, Palmfeldt J, Deficiency of the mitochondrial sulfide regulator ETHE1 disturbs cell growth, glutathione level and causes proteome alterations outside mitochondria. Biochim Biophys Acta Mol Basis Dis, 1865(1):126-135(2019) [pubmed]
10.1016/j.bbadis.2018.10.035;

Sahebekhtiari N, Fernandez-Guerra P, Nochi Z, Carlsen J, Bross P, Palmfeldt J, Deficiency of the mitochondrial sulfide regulator ETHE1 disturbs cell growth, glutathione level and causes proteome alterations outside mitochondria. Biochim Biophys Acta Mol Basis Dis, 1865(1):126-135(2019) [pubmed]

10.1016/j.bbadis.2018.10.035;

Keyword List

curator keyword: Biomedical
submitter keyword: patient fibroblasts, ETHE1 deficiency, TMT labeling,LC-MS/MS ethylmalonic acid encephalopathy

curator keyword: Biomedical

submitter keyword: patient fibroblasts, ETHE1 deficiency, TMT labeling,LC-MS/MS ethylmalonic acid encephalopathy

Contact List

Johan Palmfeldt
contact affiliation	Research Unit for Molecular Medicine, Aarhus University and University Hospital 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

Full Dataset Link List

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PRIDE project URI

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/2018/11/PXD009880

PRIDE project URI

Repository Record List

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