PXD045351 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | ETFDH, CIII and COQ2 common interactome |
Description | Proteomic analysis of the immunocapture (IP) of COQ2, ETFDH and UQCRC2 in skeletal muscle extracts, searching for common interactors Coenzyme Q (Q) is a key lipid electron transporter, but several aspects of its biosynthesis and redox homeostasis remain undefined. Various flavoproteins reduce the Q; however, in eukaryotes, only the OXPHOS-complex III (CIII) oxidizes the QH2. The mechanism of action of CIII is still debated. We demonstrate that the Q-reductase ETFDH is essential for CIII activity in skeletal muscle. We identify a complex involving ETFDH, CIII, and the Q-biosynthesis regulator COQ2 that directs electrons from lipid substrates to the respiratory chain, reducing electron leak and ROS production. This metabolon maintains Q levels, minimizes QH2-reductive stress, and improves OXPHOS efficiency. Muscle-specific ETFDH-/- mice develop myopathy due to CIII dysfunction, indicating that ETFDH is a required OXPHOS component and a potential therapeutic target for mitochondrial redox medicine |
HostingRepository | PRIDE |
AnnounceDate | 2023-10-24 |
AnnouncementXML | Submission_2023-10-24_06:25:02.496.xml |
DigitalObjectIdentifier | https://dx.doi.org/10.6019/PXD045351 |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Supported dataset by repository |
PrimarySubmitter | AlbertoParadela |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue |
Instrument | Orbitrap Exploris 240 |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2023-09-13 06:57:33 | ID requested | |
⏵ 1 | 2023-10-24 06:25:02 | announced | |
Publication List
Keyword List
submitter keyword: ETFDH |
Coenzyme Q |
OXPHOS-complex III |
skeletal muscle |
Contact List
LauraFormentini |
contact affiliation | Centro de Biologia Molecular Severo Ochoa Spanish Research Council Nicolas Cabrera,1 28049 Madrid (Spain) |
contact email | lformentini@cbm.csic.es |
lab head | |
AlbertoParadela |
contact affiliation | CNB-CSIC |
contact email | alberto.paradela@cnb.csic.es |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD045351
- Label: PRIDE project
- Name: ETFDH, CIII and COQ2 common interactome