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PXD031641

PXD031641 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleMouse heart proteomics in Oma1 and Cox10 deficient mice
DescriptionCardiomyopathy and heart failure are common manifestations in mitochondrial disease caused by deficiencies in the oxidative phosphorylation system of mitochondria (OXPHOS). Here, we demonstrate that the cardiac-specific loss of the assembly factor Cox10 of the cytochrome c oxidase causes mitochondrial cardiomyopathy in mice, which is associated with OXPHOS deficiency, lysosomal defects and an aberrant mitochondrial morphology and ultrastructure. We demonstrate activation of the mitochondrial peptidase Oma1 in Cox10-/- mice, which results in mitochondrial fragmentation and induction of the integrated stress response (ISR) along the Oma1-Dele1-Atf4 signalling axis. Ablation of Oma1 or Dele1 in Cox10-/- mice aggravates cardiomyopathy. ISR inhibition impairs the cardiac glutathione metabolism, decreases the levels of the glutathione peroxidase Gpx4 and increases lipid peroxidation in the heart, ultimately culminating in ferroptosis. Our results demonstrate a protective role of the Oma1-mediated ISR in mitochondrial cardiomyopathy and link ferroptosis to OXPHOS deficiency and mitochondrial disease.
HostingRepositoryPRIDE
AnnounceDate2022-07-27
AnnouncementXMLSubmission_2022-07-27_01:42:57.956.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterHendrik Nolte
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListNo PTMs are included in the dataset
InstrumentQ Exactive HF
Dataset History
RevisionDatetimeStatusChangeLog Entry
02022-02-13 13:23:29ID requested
12022-07-27 01:42:58announced
Publication List
Dataset with its publication pending
Keyword List
submitter keyword: Mouse, Heart, Oma, Cox10, Cardiomyopathy
Contact List
Thomas Langer
contact affiliationMax-Planck-Institute for Biology of Ageing Joseph-Stelzmann-Str. 9b 50931 Cologne, Germany
contact emailtlanger@age.mpg.de
lab head
Hendrik Nolte
contact affiliationMax Planck Institute for Biology of Ageing, 50931 Cologne, Germany
contact emailh.nolte@age.mpg.de
dataset submitter
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Dataset FTP location
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