⮝ Full datasets listing
PXD059922
PXD059922 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Increased BNIP3-mediated mitophagy attenuates GDAP1 loss of function - implications for Charcot-Marie-Tooth disease |
| Description | Charcot-Marie-Tooth (CMT) disease type 4A, an autosomal recessive neuropathy, arises from mutations in ganglioside-induced differentiation-associated protein 1 (GDAP1). GDAP1 resides in the outer mitochondrial membrane facing the cytosol and is involved in mitochondrial dynamics and function. Its perturbation affects mitochondrial shape, contact sites, redox homeostasis and cellular metabolism. In response to GDAP1 knockdown in a human neuronal cell lines, we found increased mitochondrial turnover, biogenesis, and mitophagy mediated by BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3) and its homolog BNIP3L. Flies with neural Gdap1 knockdown also exhibited upregulated levels of the sole BNIP3 ortholog. Neural expression of human BNIP3 reduced the detrimental effects of Gdap1 knockdown on eclosion and climbing ability in adult flies, while simultaneous knockdown of both genes was detrimental. These findings suggest that increased BNIP3-driven mitophagy may act as a protective mechanism, partially counteracting the cellular dysfunction caused by GDAP1 loss of function, and highlight the potential of targeting mitophagy pathways as a therapeutic strategy for CMT4A. The mitochondrial fractions of SH-SY5Y cells (GDAP1-KD and control ) were analyzed by LC-MS proteomics. |
| HostingRepository | jPOST |
| AnnounceDate | 2026-01-18 |
| AnnouncementXML | Submission_2026-01-17_07:00:05.361.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | David Gomez-Zepeda |
| SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: 9606; |
| ModificationList | S-carboxamidomethyl-L-cysteine; L-methionine sulfoxide |
| Instrument | Synapt G2-S HDMS |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2025-01-17 08:07:12 | ID requested | |
| ⏵ 1 | 2026-01-17 07:00:05 | announced |
Publication List
| Dataset with its publication pending |
Keyword List
| submitter keyword: Mitochondria, mitophagy, hereditary disease |
Contact List
| Stefan Tenzer, Axel Methner | |
|---|---|
| lab head | |
| David Gomez-Zepeda | |
| contact affiliation | HI-TRON, DKFZ |
| 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/JPST003536/ |
