000279493 001__ 279493
000279493 005__ 20260107144938.0
000279493 037__ $$aDZNE-2025-00820
000279493 1001_ $$0P:(DE-2719)2810938$$aMüller, Stephan A$$b0$$udzne
000279493 245__ $$aDataset: Proteomics of CLN3-deficient murine microglia identifies a disease associated phenotype with lysosomal alterations
000279493 260__ $$bPRoteomics IDEntifications Database$$c2024
000279493 3367_ $$2BibTeX$$aMISC
000279493 3367_ $$0PUB:(DE-HGF)32$$2PUB:(DE-HGF)$$aDataset$$bdataset$$mdataset$$s1752047323_8074
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000279493 520__ $$aLoss-of-function mutations in CLN3 cause juvenile Batten disease, featuring neurodegeneration and early-stage neuroinflammation. How loss of CLN3 function leads to early neuroinflammation is not yet understood. Here, we have comprehensively studied microglia from Cln3∆ex7/8 mice, a genetically accurate disease model. Loss of CLN3 function in microglia leads to lysosomal storage material accumulation and abnormal morphology of subcellular organelles. We also discovered pathological proteomic signatures consistent with defects in lysosomal function and indicative of abnormal lipid metabolism. CLN3-deficient microglia were unable to efficiently turnover myelin and metabolize the associated lipids, showing defects in lipid droplet formation and cholesterol accumulation.
000279493 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x0
000279493 7001_ $$0P:(DE-2719)2181459$$aLichtenthaler, Stefan$$b1$$udzne
000279493 7870_ $$0DZNE-2024-01276$$aYasa, Seda et.al.$$dLondon : Springer Nature, 2024$$iRelatedTo$$r$$tLoss of CLN3 in microglia leads to impaired lipid metabolism and myelin turnover.
000279493 8564_ $$uhttps://wwwdev.ebi.ac.uk/pride/archive/projects/PXD048550
000279493 909CO $$ooai:pub.dzne.de:279493$$pVDB
000279493 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2810938$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b0$$kDZNE
000279493 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2181459$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b1$$kDZNE
000279493 9131_ $$0G:(DE-HGF)POF4-352$$1G:(DE-HGF)POF4-350$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lNeurodegenerative Diseases$$vDisease Mechanisms$$x0
000279493 9141_ $$y2024
000279493 9201_ $$0I:(DE-2719)1110006$$kAG Lichtenthaler$$lNeuroproteomics$$x0
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000279493 980__ $$aI:(DE-2719)1110006
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