PXD046331-3

PXD046331 is an original dataset announced via ProteomeXchange.

Title	Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies
Description	Individual complexes of the mitochondrial oxidative phosphorylation system (OXPHOS) are not linked solely by their function, they also share dependencies at the maintenance/assembly level, where one complex depends on the presence of a different individual complex. Despite the relevance of this ‘interdependence’ behavior for mitochondrial diseases, its true nature remains elusive. To understand the mechanism that can explain this phenomenon, we examined the consequences of aberration of different OXPHOS complexes in human cells. We demonstrate here that complete disruption of each of OXPHOS complexes resulted in a decrease of complex I (cI) level and that the major reason for this is linked to downregulation of mitochondrial ribosomal proteins. We conclude that the secondary cI defect is due to mitochondrial protein synthesis attenuation, while the responsible signaling pathways could differ based on the origin of the OXPHOS defect.
HostingRepository	PRIDE
AnnounceDate	2025-02-18
AnnouncementXML	Submission_2025-02-18_05:58:04.761.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Marek Vrbacky
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	methylthiolated residue
Instrument	Orbitrap Exploris 480

Revision	Datetime	Status	ChangeLog Entry
0	2023-10-23 10:55:16	ID requested
1	2024-07-16 15:18:29	announced
2	2024-10-22 06:50:37	announced	2024-10-22: Updated project metadata.
⏵ 3	2025-02-18 05:58:05	announced	2025-02-18: Updated project metadata.

Č, un, á, tov, á K, Vrback, ý M, Puertas-Frias G, Al, á, n L, Vani, š, ov, á M, Saucedo-Rodr, í, guez MJ, Hou, š, t, ě, k J, Fern, á, ndez-Vizarra E, Neu, ž, il J, Pecinov, á A, Pecina P, Mr, á, č, ek T, Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies. iScience, 27(8):110560(2024) [pubmed]

10.1016/j.isci.2024.110560;

submitter keyword: integrated stress response,mitochondria, OXPHOS, complex I, mitochondrial protein synthesis, secondary deficiency, complex IV, complex V, mitochondrial translation, biogenesis interdependency

Tomas Mracek
contact affiliation	Institute of Physiology of the Czech Academy of Sciences Laboratory of Bioenergetics
contact email	tomas.mracek@fgu.cas.cz
lab head
Marek Vrbacky
contact affiliation	Czech Academy of Sciences
contact email	proteom.krc@gmail.com
dataset submitter

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/2024/07/PXD046331

PRIDE project URI

• PRIDE
  1. PXD046331
     1. Label: PRIDE project
     2. Name: Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies