PXD041783-1

PXD041783 is an original dataset announced via ProteomeXchange.

Title	Defective mitochondrial translation due to loss of COX14 function triggers ROS-induced inflammation in liver
Description	Mitochondrial oxidative phosphorylation (OXPHOS) fuels cellular ATP demands. OXPHOS defects lead to severe human disorders with unexplained tissue specific pathologies. Mitochondrial gene expression is essential for OXPHOS biogenesis since core subunits of the complexes are mitochondrial-encoded. COX14 is required for translation of COX1, the central mitochondrial-encoded subunit of complex IV. Here we generated a COX14 mouse mutant corresponding to a patient with complex IV deficiency. COX14M19I mice display broad tissue-specific pathologies. A hallmark phenotype is severe liver inflammation linked to release of mitochondrial RNA into the cytosol sensed by RIG-1 pathway. We find that mitochondrial RNA release is triggered by increased reactive oxygen species production in the absence of complex IV. Additionally, we also generated a COA3Y72C mouse, affected in COX1 biogenesis, which displays a similar yet milder inflammatory phenotype. Our study provides mechanistic insight into how defective mitochondrial gene expression causes tissue-specific inflammation.
HostingRepository	PRIDE
AnnounceDate	2024-07-25
AnnouncementXML	Submission_2024-07-25_09:52:34.602.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Svenja Victoria Kaufmann
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	No PTMs are included in the dataset
Instrument	Orbitrap Fusion

Revision	Datetime	Status	ChangeLog Entry
0	2023-04-24 10:43:17	ID requested
⏵ 1	2024-07-25 09:52:34	announced
2	2024-10-22 06:51:24	announced	2024-10-22: Updated project metadata.

Dataset with its publication pending

submitter keyword: Mouse, ROS, Liver, COX14

Henning Urlaub
contact affiliation	The Bioanalytical Mass Spectrometry Group, Max Planck Institute for Multidisciplinary Sciences, Göttingen, Germany Institute for Clinical Chemistry, University Medical Center Göttingen, Göttingen, Germany
contact email	henning.urlaub@mpinat.mpg.de
lab head
Svenja Victoria Kaufmann
contact affiliation	Max Planck Institute for Multidisciplinary Sciences
contact email	svenja.kaufmann@mpinat.mpg.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD041783
     1. Label: PRIDE project
     2. Name: Defective mitochondrial translation due to loss of COX14 function triggers ROS-induced inflammation in liver