000279494 001__ 279494
000279494 005__ 20250709100939.0
000279494 037__ $$aDZNE-2025-00821
000279494 1001_ $$0P:(DE-2719)2810938$$aMüller, Stephan A$$b0$$udzne
000279494 245__ $$aDataset: Proteomics of brain from a myeloid specific NPC1 KO mouse
000279494 260__ $$bPRoteomics IDEntifications Database$$c2024
000279494 3367_ $$2BibTeX$$aMISC
000279494 3367_ $$0PUB:(DE-HGF)32$$2PUB:(DE-HGF)$$aDataset$$bdataset$$mdataset$$s1752047338_8070
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000279494 3367_ $$2DataCite$$aDataset
000279494 3367_ $$2ORCID$$aDATA_SET
000279494 3367_ $$2DINI$$aResearchData
000279494 520__ $$aNiemann-Pick type C (NPC) disease is an inherited lysosomal storage disorder mainly driven by mutations in NPC1 gene, causing lipid accumulation within late endosomes/lysosomes, and resulting in progressive neurodegeneration. Although microglial activation proceeds neuronal loss, it remains elusive whether loss of NPC1 in microglia actively contributes to NPC pathology. Here, we used a mouse model with depletion of NPC1 in myeloid cells to investigate the role of microglia in Niemann-Pick disease. In order to achieve the loss of NPC1 in myeloid cells, mice with floxed Npc1 alleles (Npc1 flox/flox) were crossed with mice expressing the constitutively active Cre recombinase under the myeloid-specific promotor of Cx3cr1. Hyperactive microglia initiated a pathological cascade resembling NPC-like phenotypes, including shortened lifespan, motor impairments, astrogliosis, neuroaxonal pathology and increased levels of neuronal injury biomarker NF-L. To study the differential vulnerability between the brain regions, we compared the cerebellar with the cerebral (brain without cerebellum) proteome in Cre- and Cre+ mice at late pathological stages. Our results suggest that microglial loss of NPC1 has profound effects on brain cell homeostasis especially in the cerebrum.
000279494 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x0
000279494 7001_ $$0P:(DE-2719)2181459$$aLichtenthaler, Stefan$$b1$$udzne
000279494 7870_ $$0DZNE-2024-01395$$aDinkel, Lina et.al.$$dWashington, DC : AAAS, 2024$$iRelatedTo$$r$$tMyeloid cell-specific loss of NPC1 in mice recapitulates microgliosis and neurodegeneration in patients with Niemann-Pick type C disease.
000279494 8564_ $$uhttps://wwwdev.ebi.ac.uk/pride/archive/projects/PXD056188
000279494 909CO $$ooai:pub.dzne.de:279494$$pVDB
000279494 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2810938$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b0$$kDZNE
000279494 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2181459$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b1$$kDZNE
000279494 9131_ $$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$$x0
000279494 9201_ $$0I:(DE-2719)1110006$$kAG Lichtenthaler$$lNeuroproteomics$$x0
000279494 980__ $$adataset
000279494 980__ $$aVDB
000279494 980__ $$aI:(DE-2719)1110006
000279494 980__ $$aUNRESTRICTED