PXD031641 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Mouse heart proteomics in Oma1 and Cox10 deficient mice |
Description | Cardiomyopathy 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. |
HostingRepository | PRIDE |
AnnounceDate | 2022-07-27 |
AnnouncementXML | Submission_2022-07-27_01:42:57.956.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Hendrik Nolte |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | No PTMs are included in the dataset |
Instrument | Q Exactive HF |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2022-02-13 13:23:29 | ID requested | |
⏵ 1 | 2022-07-27 01:42:58 | announced | |
Publication List
Dataset with its publication pending |
Keyword List
submitter keyword: Mouse, Heart, Oma, Cox10, Cardiomyopathy |
Contact List
Thomas Langer |
contact affiliation | Max-Planck-Institute for Biology of Ageing Joseph-Stelzmann-Str. 9b 50931 Cologne, Germany |
contact email | tlanger@age.mpg.de |
lab head | |
Hendrik Nolte |
contact affiliation | Max Planck Institute for Biology of Ageing, 50931 Cologne, Germany |
contact email | h.nolte@age.mpg.de |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD031641
- Label: PRIDE project
- Name: Mouse heart proteomics in Oma1 and Cox10 deficient mice