⮝ Full datasets listing
PXD068316
PXD068316 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Accumulated mtDNA mutations specifically impair complex I-linked respiration |
| Description | Oxidative phosphorylation (OxPhos) within mitochondria relies on the coordinated synthesis of both nuclear- and mitochondrial-encoded protein subunits comprising the mitochondrial respiratory complexes (Complexes I–V). Mitochondrial DNA (mtDNA) encodes 13 proteins vital for Complex I, III, IV, and V function. Accumulated mutations in mtDNA are causally linked to aging and several age-related diseases, presumably as a consequence of impaired respiration. However, how high mtDNA mutation burden impinges on cellular bioenergetics across the major organ systems remains only partially resolved. Due to the growing links connecting mtDNA mutations to human pathophysiology, here we leveraged a comprehensive mitochondrial phenotyping platform to assess the phenotypic consequences of increased mtDNA mutation burden across tissues (brown adipose, brain, colon, heart, kidney, liver, lung, and bone marrow-derived mononuclear cells) of the mouse. Remarkably, despite widespread reductions in OxPhos protein expression, mitochondrial respiratory capacity under mixed substrate conditions was largely preserved across tissues. More detailed analysis revealed that NAD-linked respiration exhibited partial functional deficits in most tissues, consistent with functional deficiencies in complex I. In contrast, respiration routed from CII-CIII-CIV-CV remained intact. Together, these findings highlight Complex I as the primary functional consequence of mtDNA mutational load. |
| HostingRepository | jPOST |
| AnnounceDate | 2026-02-19 |
| AnnouncementXML | Submission_2026-02-19_13:43:31.153.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Kelsey Fisher-Wellman |
| SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
| ModificationList | S-carboxamidomethyl-L-cysteine; L-methionine sulfoxide |
| Instrument | Q Exactive |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2025-09-12 06:20:03 | ID requested | |
| ⏵ 1 | 2026-02-19 13:43:31 | announced |
Publication List
| Dataset with its publication pending |
Keyword List
| submitter keyword: PolG, mitochondria, complex I |
Contact List
| Kelsey H Fisher-Wellman | |
|---|---|
| lab head | |
| Kelsey Fisher-Wellman | |
| contact affiliation | Wake Forest University |
| dataset submitter | |
Full Dataset Link List
| jPOST 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://ftp.jpostdb.org/JPST004072/ |
