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PXD009743-1

PXD009743 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleInsights into the pathological mechanisms of MT-ND5 and NDUFS1 mutation in mitochondrial complex I by proteome and metabolome profiling
DescriptionIn 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.
HostingRepositoryPRIDE
AnnounceDate2019-09-30
AnnouncementXMLSubmission_2019-09-30_03:39:56.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterYang Ni
SpeciesList scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationListmonohydroxylated residue; acetylated residue; iodoacetamide derivatized residue
InstrumentQ Exactive Plus
Dataset History
RevisionDatetimeStatusChangeLog Entry
02018-05-10 23:50:44ID requested
12019-09-30 03:39:57announced
22019-10-16 04:09:45announced2019-10-16: Updated publication reference for PubMed record(s): 31557978.
32024-10-22 04:58:02announced2024-10-22: 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 affiliationMass Spectrometry Facility Max Planck Institute for Molecular Genetics Ihnestrasse 63-73 14195 Berlin GERMANY
contact emailmeierhof@molgen.mpg.de
lab head
Yang Ni
contact affiliationMax Planck Institute for Molecular Genetics
contact emailni@molgen.mpg.de
dataset submitter
Full Dataset Link List
Dataset FTP location
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PRIDE project URI
Repository Record List
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