GEO DataSets

(Submitter supplied) Here, we report the characterization and the comparison of the transcriptomes of BV-2 murine microglial mutant cell lines (CRISPR/Cas9-edited mutations in peroxisomal genes) by RNA-sequencing. Microglia is suspected to play a major role in the neurodegenerative processes observed in peroxisomal leukodystrophies. From CRISPR/Cas9-edited BV-2 microglial cell lines, we aimed at exploring the transcriptomic consequences of a defect of peroxisomal beta-oxidation.

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL21103

15 Samples

Download data: TSV

(Submitter supplied) In X-linked adrenoleukodystrophy (X-ALD), the most common peroxisomal disorder, microglial defect is suggested to prime and amplify the neuroinflammatory process. By using CRISPR/Cas9 gene editing, we recently established BV-2 microglial cell models to study the impact of dysfunctional peroxisomal b-oxidation and demonstrated the emergence of a disease-associated microglial signature in these cell lines. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL21103

18 Samples

Download data: TSV

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL11154

25 Samples

Download data

(Submitter supplied) CD14+ monocytes were isolated from the blood of healthy human donors by Ficoll density-gradient centrifugation, magnetic cell sorting and LS column systems. CD14+ monocytes were in vitro differentiated to macrophages by culturing the cells for 7 days in RPMI medium containing 10% FCS, 1% penicillin/streptomycin, 1% glutamine, 1% Fungizone and supplemented with 50 ng/ml human macrophage-colony stimulating factor (M-CSF) in the presence or absence of myelin isolated from brain tissue of 6-8 week-old wild-type C57BL6/J mice.

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL11154

8 Samples

Download data: TXT

(Submitter supplied) Saturated very long-chain fatty acids (VLCFA, ≥ C22), enriched in brain myelin and innate immune cells, accumulate in X-linked adrenoleukodystrophy (X-ALD). The severest form, inherited dysfunction of the VLCFA transporter ABCD1, underlying X-ALD, causes brain myelin destruction with infiltration of pro-inflammatory skewed monocytes/macrophages. How VLCFA levels relate to macrophage activation is unclear. more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL11154

17 Samples

Download data: TSV

(Submitter supplied) Purpose: We purified whole brain microglia of MFP2 knockout mice and control mice utilizing percoll gradient and FACS sorting, followed by microarray analysis to define the molecular changes in MFP2 knockout mice at the endstage of the disease. We compared the microglia transcriptome of Mfp2-/- microglia to that of SOD1-G93A microglia isolated from spinal cord to define the microglia signature associated with a non-neurodegenerative environment. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Platform:

GPL6246

8 Samples

Download data: CEL

(Submitter supplied) Microglia-mediated neuroinflammation has been implicated in the pathogenesis of Alzheimer's disease (AD). Although microglia in aging and neurodegenerative disease model mice show a loss of homeostatic phenotype and activation of disease-associated microglia (DAM), a correlation between those phenotypes and the degree of neuronal cell loss has not been clarified. In this study, we performed RNA sequencing of microglia isolated from three representative neurodegenerative mouse models, AppNL-G-F/NL-G-F with amyloid pathology, rTg4510 with tauopathy, and SOD1G93A with motor neuron disease by magnetic activated cell sorting. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platforms:

GPL24247 GPL21273

22 Samples

Download data: XLSX

(Submitter supplied) Although not an affected cell type, skin fibroblasts from individuals with CC-ALD, an early onset X-linked neurological disorder, show defects in very long chain fatty acid (VLCFA) metabolism that provide the basis for clinical diagnostic tests. Skin fibroblasts from CC-ALD patients can be reprogrammed into iPS cells with all the hallmark properties of pluripotency. The iPS cell phenotypes may reflect the tissue-specificity of the lipid metabolic defects found in CC-ALD patients. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Dataset:

GDS4451

Platform:

GPL571

18 Samples

Download data: CEL

(Submitter supplied) Although not an affected cell type, skin fibroblasts from individuals with childhood cerebral adrenoleukodystrophy (CCALD), an early onset X-linked neurological disorder, show defects in very long chain fatty acid (VLCFA) metabolism that provide the basis for clinical diagnostic tests. We report the gene expression profiles of fibroblasts from childhood cerebral adrenoleukodystrophy patients and healthy controls

Organism:

Homo sapiens

Type:

Expression profiling by array

Dataset:

GDS4559

Platform:

GPL570

10 Samples

Download data: CEL

Analysis of fibroblasts from patients with CCALD, the childhood cerebral form of X-linked adrenoleukodystrophy (X-ALD). X-ALD is an early onset X-linked neurological disorder affecting the nervous, adrenocortical, and male reproductive systems. Results provide insight into molecular basis of CCALD.

Organism:

Homo sapiens

Type:

Expression profiling by array, transformed count, 2 disease state sets

Platform:

GPL570

Series:

GSE34308

10 Samples

Download data: CEL

Analysis of induced pluripotent stem cells (iPSC) derived from childhood cerebral adrenoleukodystrophy (CCALD) patient dermal fibroblasts. Inflammatory brain demyelination is observed in CCALD patients. Results provide insight into molecular mechanisms underlying CCALD pathogenesis.

Organism:

Homo sapiens

Type:

Expression profiling by array, transformed count, 2 cell type, 2 disease state sets

Platform:

GPL571

Series:

GSE34309

18 Samples

Download data: CEL