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| 001 | 279494 | ||
| 005 | 20250709100939.0 | ||
| 037 | _ | _ | |a DZNE-2025-00821 |
| 100 | 1 | _ | |a Müller, Stephan A |0 P:(DE-2719)2810938 |b 0 |u dzne |
| 245 | _ | _ | |a Dataset: Proteomics of brain from a myeloid specific NPC1 KO mouse |
| 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 1752047338_8070 |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 Niemann-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. |
| 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 Dinkel, Lina et.al. |d Washington, DC : AAAS, 2024 |i RelatedTo |0 DZNE-2024-01395 |r |t Myeloid cell-specific loss of NPC1 in mice recapitulates microgliosis and neurodegeneration in patients with Niemann-Pick type C disease. |
| 856 | 4 | _ | |u https://wwwdev.ebi.ac.uk/pride/archive/projects/PXD056188 |
| 909 | C | O | |o oai:pub.dzne.de:279494 |p VDB |
| 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 |b 1 |6 P:(DE-2719)2181459 |
| 913 | 1 | _ | |a DE-HGF |b Gesundheit |l Neurodegenerative Diseases |1 G:(DE-HGF)POF4-350 |0 G:(DE-HGF)POF4-352 |3 G:(DE-HGF)POF4 |2 G:(DE-HGF)POF4-300 |4 G:(DE-HGF)POF |v Disease Mechanisms |x 0 |
| 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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