PXD070104 is an
original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Identification of novel substrates of the human mitochondrial ClpXP protease: Implications to Perrault syndrome type 3 |
| Description | The ClpXP ATP-dependent unfoldase-protease complex plays important roles in maintaining protein homeostasis in the mitochondria. Pathogenic variants of CLPP have been identified in patients with Perrault syndrome type 3 (PRLTS3), a rare recessive mitochondrial disease characterized by sensorineural hearing loss, ovarian dysfunction, and neurological abnormalities. We performed proteomic analysis (DIA acquisition) of PRLTS3 patient-derived fibroblasts and in conjunction with other experiments, identified three novel substrates of ClpXP. |
| HostingRepository | PRIDE |
| AnnounceDate | 2026-08-13 |
| AnnouncementXML | Submission_2026-08-13_06:15:14.789.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Matthias Trost |
| SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606; |
| ModificationList | monohydroxylated residue |
| Instrument | timsTOF HT |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|
| 0 | 2025-10-30 08:19:02 | ID requested | |
| ⏵ 1 | 2026-08-13 06:15:15 | announced | |
Publication List
| Dataset with its publication pending |
Keyword List
| submitter keyword: Human, timsTOF HT |
Contact List
| Matthias Trost |
|---|
| contact affiliation | Laboratory for Biomedical Mass Spectrometry, Biosciences Institute, Newcastle University, Newcastle upon Tyne, UK. |
| contact email | matthias.trost@newcastle.ac.uk |
| lab head | |
| Matthias Trost |
|---|
| contact affiliation | Newcastle University |
| contact email | matthias.trost@ncl.ac.uk |
| dataset submitter | |
Full Dataset Link List
Dataset FTP location
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| PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD070104
- Label: PRIDE project
- Name: Identification of novel substrates of the human mitochondrial ClpXP protease: Implications to Perrault syndrome type 3
