PXD009743 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Insights into the pathological mechanisms of MT-ND5 and NDUFS1 mutation in mitochondrial complex I by proteome and metabolome profiling |
Description | In this study, we used cell cultures of skin fibroblasts from two patients carrying missense mutations in the core subunit MT-ND5 and NDUFS1 of complex I (CI), respectively. We applied quantitative proteome- and metabolome profiling together with cell respiration assays and conventional biochemical methods to dissect the underlying mechanisms of CI dysfunction. Label-free quantification (LFQ) proteome profiling showed that the N-module of CI was severely downregulated in the NDUFS1 patient, indicating a disassembly of CI, which was further confirmed by Blue Native PAGE (BN PAGE) and Western Blot. |
HostingRepository | PRIDE |
AnnounceDate | 2019-09-30 |
AnnouncementXML | Submission_2019-09-30_03:39:56.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Yang Ni |
SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: 9606; |
ModificationList | monohydroxylated residue; acetylated residue; iodoacetamide derivatized residue |
Instrument | Q Exactive Plus |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2018-05-10 23:50:44 | ID requested | |
⏵ 1 | 2019-09-30 03:39:57 | announced | |
2 | 2019-10-16 04:09:45 | announced | 2019-10-16: Updated publication reference for PubMed record(s): 31557978. |
3 | 2024-10-22 04:58:02 | announced | 2024-10-22: Updated project metadata. |
Publication List
Dataset with its publication pending |
Keyword List
curator keyword: Biomedical |
submitter keyword: Mitochondrial complex I, MT-ND5, NDUFS1, proteome profiling, metabolome profiling |
Contact List
David Meierhofer |
contact affiliation | Mass Spectrometry Facility Max Planck Institute for Molecular Genetics Ihnestrasse 63-73 14195 Berlin GERMANY |
contact email | meierhof@molgen.mpg.de |
lab head | |
Yang Ni |
contact affiliation | Max Planck Institute for Molecular Genetics |
contact email | ni@molgen.mpg.de |
dataset submitter | |
Full Dataset Link List
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/2019/09/PXD009743 |
PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD009743
- Label: PRIDE project
- Name: Insights into the pathological mechanisms of MT-ND5 and NDUFS1 mutation in mitochondrial complex I by proteome and metabolome profiling