PXD044146
PXD044146 is an original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Longitudinal Deep Multi-Omics Profiling in a CLN3 Minipig Model Reveals Novel Biomarker Signatures for Batten Disease |
| Description | Development of therapies for CLN3 Batten disease, a rare pediatric lysosomal storage disorder, has been hindered by a lack of etiological insights and translatable biomarkers. Here, we used deep multi-omics to discover new biomarkers using longitudinal serum samples from a porcine model of CLN3 disease, which was previously impossible due to lack of efficient serum proteome profiling technologies compatible with model organisms. Comprehensive metabolomics was combined with a nanoparticle-based LC-MS-based proteomic profiling coupled with TMTpro 18-plex to generate quantitative data on 769 metabolites and 2,634 proteins, collectively the most exhaustive multi-omics profile conducted on serum from a porcine model. The presymptomatic disease state was characterized by elevations in glycerophosphodiester species and lysosomal proteases, while later timepoints were enriched with species involved in immune cell activation and sphingolipid metabolism. Cathepsin S, Cathepsin B, glycerophosphoinositol, and glycerophosphoethanolamine captured a large portion of the genotype-correlated variation between healthy and diseased animals, suggesting that an index score based on these analytes could have great utility for tracking disease in the clinic. |
| HostingRepository | MassIVE |
| AnnounceDate | 2025-03-10 |
| AnnouncementXML | Submission_2025-03-10_21:42:06.138.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Non peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Alexandre Rosa Campos |
| SpeciesList | scientific name: Sus scrofa; common name: pig; NCBI TaxID: 9823; |
| ModificationList | Oxidation; TMTpro |
| Instrument | Orbitrap Fusion Lumos |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|---|---|---|
| 0 | 2023-07-27 09:54:07 | ID requested | |
| ⏵ 1 | 2025-03-10 21:42:06 | announced |
Publication List
| no publication |
Keyword List
| submitter keyword: sphingolipid metabolism, lysosomal storage, blood serum, lipidomics, metabolomics |
Contact List
| Jon J Brudvig | |
|---|---|
| contact affiliation | Sanford Research |
| contact email | jon.brudvig@sanfordhealth.org |
| lab head | |
| Alexandre Rosa Campos | |
| contact affiliation | SBP Medical Discovery Institute |
| contact email | arosacampos@sbpdiscovery.org |
| 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/v06/MSV000092536/ |
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