PXD045352

PXD045352 is an original dataset announced via ProteomeXchange.

Title	ETFDH comigrates with CIII in BN-PAGE
Description	Proteomic analysis of 13 bands from BN-PAGE immunostaining of mouse skeletal muscle mitochondrial proteins: 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_06:26:58.765.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD045352
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

Revision	Datetime	Status	ChangeLog Entry
0	2023-09-13 07:14:52	ID requested
⏵ 1	2023-10-24 06:26:59	announced

10.6019/PXD045352;

submitter keyword: Coenzyme Q

OXPHOS-complex III

skeletal muscle

mitochondria

LauraFormentini
contact affiliation	Centro de Biología 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

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PRIDE project URI

• PRIDE
  1. PXD045352
     1. Label: PRIDE project
     2. Name: ETFDH comigrates with CIII in BN-PAGE