Homo sapiens
Ontology highlight
ABSTRACT:
PROVIDER: PRJNA102055 | ENA |
REPOSITORIES: ENA
Similar Datasets
Project description:Purkinje cells (PC) of the cerebellum degenerate in adult mice with mutations in the Niemann-Pick type C (NPC) disease 1 (Npc1) gene. We subjected BALB/c Npc1+/+ and Npc1-/- mouse cerebella from an early and a later time point of PC degeneration to a genome-wide microarray gene expression analysis. We found general underrepresentation of PC-specific transcripts, consistent with PC loss, and elevated markers of microglia activation at the later time point. Keywords: Niemann-Pick type C, Purkinje cell degeneration
2007-07-01 | GSE5944 | GEO
Project description:Macrophage inflammatory protein 1alpha/CCL3 protein is a known pro-inflammatory cytokine that can mediate chemotaxis of monocytes and promote cell degranulation. Ccl3 gene expression is elevated in the CNS and visceral tissue of many lysosomal storage disorders. The deletion of Ccl3 in a mouse model of Sandhoff disease was reported to result in reduced monocyte-associated pathology in the brain, delayed neurodegeneration, and prolonged health. However, deletion of Ccl3 in a mouse model of Niemann-Pick C disease was dentrimental or neutral instead of beneficial. Prevention of neuronal loss was instead mediated by providing NPC1 to neurons. We used microarrays to detail the global change in gene expression of the cerebellum in Niemann-Pick C disease animals, Niemann-Pick C disease animals with Ccl3 gene deletion, and Niemann-Pick C disease animals with Purkinje neuron-specific NPC1-YFP rescue.
2012-04-17 | GSE36119 | GEO
Project description:Untargeted lipidomic analysis via QTOF and FAME analysis by GC-EI-MS in Niemann-pick type C1
2019-07-16 | MSV000084097 | MassIVE
Project description:Macrophage inflammatory protein 1alpha/CCL3 protein is a known pro-inflammatory cytokine that can mediate chemotaxis of monocytes and promote cell degranulation. Ccl3 gene expression is elevated in the CNS and visceral tissue of many lysosomal storage disorders. The deletion of Ccl3 in a mouse model of Sandhoff disease was reported to result in reduced monocyte-associated pathology in the brain, delayed neurodegeneration, and prolonged health. However, deletion of Ccl3 in a mouse model of Niemann-Pick C disease was dentrimental or neutral instead of beneficial. Prevention of neuronal loss was instead mediated by providing NPC1 to neurons. We used microarrays to detail the global change in gene expression of the cerebellum in Niemann-Pick C disease animals, Niemann-Pick C disease animals with Ccl3 gene deletion, and Niemann-Pick C disease animals with Purkinje neuron-specific NPC1-YFP rescue. To identify the top ~50 genes elevated in NPC disease Npc1-/- (NPC) and Npc1+/- (WT) mice were compared at age P50; To profile changes in gene expression as a result of Ccl3 gene deletion Ccl3-/-;Npc1-/- mice were compared against Npc1-/- mice across various ages; To profile changes in gene expression as a result of Purkinje neuron-sepcific NPC1 rescue P;N;Npc1-/- mice were compared against Npc1-/- mice across various ages.
2012-04-16 | E-GEOD-36119 | biostudies-arrayexpress
Project description:Untargeted lipidomic analysis via QTOF and FAME analysis by GC-EI-MS in Niemann-pick type C1
2019-07-16 | MSV000084097 | GNPS
Project description:Drosophila melanogaster Npc2c, Niemann-Pick type C-2c, is differentially expressed in 10 experiment(s);
| FBgn0037783 | ExpressionAtlas
Project description:Drosophila melanogaster Npc2d, Niemann-Pick type C-2d, is differentially expressed in 12 experiment(s);
| FBgn0037782 | ExpressionAtlas
Project description:Drosophila melanogaster Npc2h, Niemann-Pick type C-2h, is differentially expressed in 18 experiment(s);
| FBgn0039801 | ExpressionAtlas
Project description:Drosophila melanogaster Npc2f, Niemann-Pick type C-2f, is differentially expressed in 13 experiment(s);
| FBgn0039154 | ExpressionAtlas
Project description:Drosophila melanogaster Npc2e, Niemann-Pick type C-2e, is expressed in 2 baseline experiment(s);
| FBgn0051410 | ExpressionAtlas