PXD048550 is an
original dataset announced via ProteomeXchange.
Dataset Summary
Title | Proteomics of CLN3-deficient murine microglia identifies a disease associated phenotype with lysosomal alterations |
Description | Loss-of-function mutations in CLN3 cause juvenile Batten disease, featuring neurodegeneration and early-stage neuroinflammation. How loss of CLN3 function leads to early neuroinflammation is not yet understood. Here, we have comprehensively studied microglia from Cln3∆ex7/8 mice, a genetically accurate disease model. Loss of CLN3 function in microglia leads to lysosomal storage material accumulation and abnormal morphology of subcellular organelles. We also discovered pathological proteomic signatures consistent with defects in lysosomal function and indicative of abnormal lipid metabolism. CLN3-deficient microglia were unable to efficiently turnover myelin and metabolize the associated lipids, showing defects in lipid droplet formation and cholesterol accumulation. |
HostingRepository | PRIDE |
AnnounceDate | 2024-10-23 |
AnnouncementXML | Submission_2024-10-23_01:27:33.854.xml |
DigitalObjectIdentifier | |
ReviewLevel | Peer-reviewed dataset |
DatasetOrigin | Original dataset |
RepositorySupport | Unsupported dataset by repository |
PrimarySubmitter | Stephan Mueller |
SpeciesList | scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; |
ModificationList | acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue |
Instrument | Q Exactive HF |
Dataset History
Revision | Datetime | Status | ChangeLog Entry |
0 | 2024-01-15 02:37:45 | ID requested | |
⏵ 1 | 2024-10-23 01:27:34 | announced | |
Publication List
Keyword List
submitter keyword: neuronal ceroid lipofuscinoses, lysosomal storage disorder,Cln3∆ex7/8, mouse, microglia, juvenile Batten disease, CLN3 |
Contact List
Stefan F. Lichtenthaler |
contact affiliation | DZNE Munich, Neuroproteomics, Feodor-Lynen Str. 17, 81377 Munich, Germany |
contact email | stefan.lichtenthaler@dzne.de |
lab head | |
Stephan Mueller |
contact affiliation | DZNE Munich Neuroproteomics |
contact email | stephan.mueller@dzne.de |
dataset submitter | |
Full Dataset Link List
Dataset FTP location
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PRIDE project URI |
Repository Record List
[ + ]
[ - ]
- PRIDE
- PXD048550
- Label: PRIDE project
- Name: Proteomics of CLN3-deficient murine microglia identifies a disease associated phenotype with lysosomal alterations