PXD081638 is an
original dataset announced via ProteomeXchange.
Dataset Summary
| Title | Batten disease protein CLN8 enables a non-canonical phospholipid synthesis pathway |
| Description | According to our current knowledge, de novo glycerophospholipid (GPL) synthesis begins with the acylation of glycerol-3-phosphate to produce phosphatidic acid, the precursor of all other GPLs. Here we describe an alternative GPL synthesis pathway that starts with the acyl-CoA-dependent acylation of glycerophosphoglycerol (GPG), resulting in the formation of lysophosphatidylglycerol (LPG). The acyltransferase reaction is catalyzed by the Batten disease-associated protein ceroid lipofuscinosis neuronal 8 (CLN8). Tracer studies revealed that CLN8-derived LPG is selectively converted into bis(monoacylglycero)phosphate (BMP), a GPL essential for lysosomal lipid homeostasis, but not into phosphatidylglycerol and cardiolipin. CLN8-knockout cells and mice cannot utilize GPG for BMP synthesis, resulting in BMP-deficiency and excess accumulation of phospholipids in lysosomes. The lipid synthesis pathway described herein is relevant for understanding lysosomal lipid metabolism and the pathogenesis of neurodegenerative diseases. |
| HostingRepository | PRIDE |
| AnnounceDate | 2026-08-04 |
| AnnouncementXML | Submission_2026-08-04_03:36:06.796.xml |
| DigitalObjectIdentifier | |
| ReviewLevel | Peer-reviewed dataset |
| DatasetOrigin | Original dataset |
| RepositorySupport | Unsupported dataset by repository |
| PrimarySubmitter | Natalia Kunowska |
| SpeciesList | scientific name: Homo sapiens (Human); NCBI TaxID: NEWT:9606; |
| ModificationList | Oxidation; acetylated residue |
| Instrument | timsTOF Pro |
Dataset History
| Revision | Datetime | Status | ChangeLog Entry |
|---|
| 0 | 2026-07-24 18:37:58 | ID requested | |
| ⏵ 1 | 2026-08-04 03:36:07 | announced | |
| 2 | 2026-08-06 08:02:54 | announced | 2026-08-06: Updated project metadata. |
Publication List
Keyword List
| submitter keyword: glycerophospholipid (GPL) synthesis |
Contact List
| Ulrich Stelzl |
|---|
| contact affiliation | Network Pharmacology, Institute of Pharmaceutical Sciences, University of Graz |
| contact email | ulrich.stelzl@uni-graz.at |
| lab head | |
| Natalia Kunowska |
|---|
| contact affiliation | Institute of Pharmaceutical Sciences
University of Graz |
| contact email | natalia.kunowska@uni-graz.at |
| dataset submitter | |
Full Dataset Link List
Dataset FTP location
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| PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD081638
- Label: PRIDE project
- Name: Batten disease protein CLN8 enables a non-canonical phospholipid synthesis pathway
