PXD042788-1
PXD042788 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Metabolic switch from fatty acid oxidation to glycolysis in knock-in mouse model of Barth Syndrome. |
| Description | Mitochondria are central for cellular metabolism and energy supply. Barth Syndrome (BTHS) is a severe disorder, due to dysfunction of the mitochondrial cardiolipin acyl transferase tafazzin. Altered cardiolipin remodeling affects mitochondrial inner membrane organization and function of membrane proteins such as transporters and the oxidative phosphorylation (OXPHOS) system. Here, we describe a mouse model that carries a G197V exchange in tafazzin, corresponding to BTHS patient. TAZG197V mice recapitulate disease-specific pathology including cardiac dysfunction and reduced oxidative phosphorylation. We show that mutant mitochondria display defective fatty acid driven oxidative phosphorylation due to reduced levels of carnitine palmitoyl transferases. A metabolic switch in ATP production from OXPHOS to glycolysis is apparent in mouse heart and patient iPSC cell-derived cardiomyocytes. An increase in glycolytic ATP production inactivates AMPK causing altered metabolic signaling in TAZG197V. Treatment of mutant cells with AMPK activator reestablishes fatty acid driven OXPHOS and protects mice against cardiac failure. |
| HostingRepository | MassIVE |
| AnnounceDate | 2023-07-17 |
| AnnouncementXML | Submission_2023-07-17_06:50:52.657.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Svenja Kaufmann |
| SpeciesList | scientific name: Mus musculus; common name: house mouse; NCBI TaxID: 10090; |
| ModificationList | No PTMs are included in the dataset |
| Instrument | Orbitrap Fusion |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2023-06-07 02:59:12 | ID requested | |
| ⏵ 1 | 2023-07-17 06:50:53 | announced |
Publication List
| no publication |
Keyword List
| submitter keyword: Barth Syndrome, Mus musculus, Fatty acid oxidation |
Contact List
| Peter Rehling | |
|---|---|
| contact affiliation | Department of Cellular Biochemistry, University Medical Center Goettingen, 37073 Goettingen, Germany |
| contact email | peter.rehling@medizin.uni-goettingen.de |
| lab head | |
| Svenja Kaufmann | |
| contact affiliation | Max Planck Institute for Multidisciplinary Sciences |
| contact email | svenja.kaufmann@mpinat.mpg.de |
| dataset submitter | |
Full Dataset Link List
| MassIVE dataset URI |
| 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://massive.ucsd.edu/MSV000092116/ |
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