PXD023881 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Pyruvate kinase supports the metabolic shift and long-term survival of a mouse model with mitochondrial dysfunction caused by DGUOK deficiency |
Description | The deoxyguanosine kinase (DGUOK) deficiency causes mtDNA depletion and mitochondrial dysfunction. We reported prolonged survival of Dguok knockout (Dguok-/-) mice despite low (<5%) mtDNA content in the liver. However, the molecular mechanisms, enabling the extended survival, remain unknown. Using transcriptomics, proteomics and metabolomics followed by in vitro assays, we aimed to identify the molecular pathways involved in the extended survival of Dguok-/- mice. At the early stage, the serine synthesis and folate cycle were activated but declined later. Increased activity of the mitochondrial citric acid cycle (TCA cycle) and the urea cycle and degradation of branched amino acids were hallmarks of the extended lifespan in DGUOK-deficiency. Furthermore, the increased synthesis of TCA cycle intermediates was supported by coordination of two pyruvate kinase genes, Pklr and Pkm, indicating a central coordinating role of pyruvate kinases to support the long-term survival in mitochondrial dysfunction. |
HostingRepository | PRIDE |
AnnounceDate | 2022-02-16 |
AnnouncementXML | Submission_2022-02-16_14:05:35.002.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Akos Vegvari |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | iodoacetamide derivatized residue |
Instrument | LTQ FT |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2021-01-29 01:29:53 | ID requested | |
⏵ 1 | 2022-02-16 14:05:36 | announced | |
Publication List
Zhou X, Mikaeloff F, Curbo S, Zhao Q, Kuiper R, V, é, gv, á, ri Á, Neogi U, Karlsson A, Coordinated pyruvate kinase activity is crucial for metabolic adaptation and cell survival during mitochondrial dysfunction. Hum Mol Genet, 30(21):2012-2026(2021) [pubmed] |
Keyword List
submitter keyword: Mouse, Label-free quantification, TMT-based quantification, DGUOK deficiency |
Contact List
Akos Vegvari |
contact affiliation | Division of Chemistry I, Department of Medical Biochemistry & Biphysics, Karolinska Institutet, Stockholm, Sweden |
contact email | akos.vegvari@ki.se |
lab head | |
Akos Vegvari |
contact affiliation | Karolinska Institutet |
contact email | akos.vegvari@ki.se |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD023881
- Label: PRIDE project
- Name: Pyruvate kinase supports the metabolic shift and long-term survival of a mouse model with mitochondrial dysfunction caused by DGUOK deficiency