Home > Publications Database > Dataset: Proteomics of CLN3-deficient murine microglia identifies a disease associated phenotype with lysosomal alterations > print

001     279493
005     20260107144938.0
037 _ _ |a DZNE-2025-00820
100 1 _ |a Müller, Stephan A
|0 P:(DE-2719)2810938
|b 0
|u dzne
245 _ _ |a Dataset: Proteomics of CLN3-deficient murine microglia identifies a disease associated phenotype with lysosomal alterations
260 _ _ |c 2024
|b PRoteomics IDEntifications Database
336 7 _ |a MISC
|2 BibTeX
336 7 _ |a Dataset
|b dataset
|m dataset
|0 PUB:(DE-HGF)32
|s 1752047323_8074
|2 PUB:(DE-HGF)
336 7 _ |a Chart or Table
|0 26
|2 EndNote
336 7 _ |a Dataset
|2 DataCite
336 7 _ |a DATA_SET
|2 ORCID
336 7 _ |a ResearchData
|2 DINI
520 _ _ |a 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.
536 _ _ |a 352 - Disease Mechanisms (POF4-352)
|0 G:(DE-HGF)POF4-352
|c POF4-352
|f POF IV
|x 0
700 1 _ |a Lichtenthaler, Stefan
|0 P:(DE-2719)2181459
|b 1
|u dzne
787 0 _ |a Yasa, Seda et.al.
|d London : Springer Nature, 2024
|i RelatedTo
|0 DZNE-2024-01276
|r
|t Loss of CLN3 in microglia leads to impaired lipid metabolism and myelin turnover.
856 4 _ |u https://wwwdev.ebi.ac.uk/pride/archive/projects/PXD048550
909 C O |o oai:pub.dzne.de:279493
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
|k DZNE
|b 0
|6 P:(DE-2719)2810938
910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
|k DZNE
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|6 P:(DE-2719)2181459
913 1 _ |a DE-HGF
|b Gesundheit
|l Neurodegenerative Diseases
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|0 G:(DE-HGF)POF4-352
|3 G:(DE-HGF)POF4
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|v Disease Mechanisms
|x 0
914 1 _ |Y 2024
920 1 _ |0 I:(DE-2719)1110006
|k AG Lichtenthaler
|l Neuroproteomics
|x 0
980 _ _ |a dataset
980 _ _ |a VDB
980 _ _ |a I:(DE-2719)1110006
980 _ _ |a UNRESTRICTED

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