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@MISC{Mller:279494,
      author       = {Müller, Stephan A and Lichtenthaler, Stefan},
      title        = {{D}ataset: {P}roteomics of brain from a myeloid specific
                      {NPC}1 {KO} mouse},
      publisher    = {PRoteomics IDEntifications Database},
      reportid     = {DZNE-2025-00821},
      year         = {2024},
      abstract     = {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.},
      cin          = {AG Lichtenthaler},
      cid          = {I:(DE-2719)1110006},
      pnm          = {352 - Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)32},
      url          = {https://pub.dzne.de/record/279494},
}