Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Transcriptome of human primary macrophages from X-linked adrenoleukodystrophy patients and healthy controls

ABSTRACT: Transcriptome of human primary macrophages from X-linked adrenoleukodystrophy patients and healthy controls

PROVIDER: PRJNA896999 | ENA |

REPOSITORIES: ENA

ACCESS DATA

Json Xml

Dataset's files

Source:

			Action	DRS
	SRR22148778.fastq.gz	Fastqsanger.gz
	SRR22148779.fastq.gz	Fastqsanger.gz
	SRR22148780.fastq.gz	Fastqsanger.gz
	SRR22148781.fastq.gz	Fastqsanger.gz
	SRR22148782.fastq.gz	Fastqsanger.gz

Items per page:

1 - 5 of 17

Similar Datasets

Transcriptome of human primary macrophages from X-linked adrenoleukodystrophy patients and healthy controls

Project description: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. Using whole transcriptome sequencing of X-ALD macrophages, we revealed that VLCFAs prime human macrophage membranes for inflammation and increase factors involved in chemotaxis and invasion. When applied externally, mimicking lipid destruction in X-ALD lesions, VLCFAs did not activate toll-like receptors in healthy cells but provoked pro-inflammatory responses through scavenger receptor CD36-mediated uptake, cumulating in JNK signalling and expression of matrix degrading enzymes and chemokine release. Following pro-inflammatory LPS-activation, VLCFA accumulated in healthy macrophages but were rapidly cleared with onset of resolution by increasing VLCFA degradation through liver-X-receptor mediated upregulation of ABCD1. ABCD1 deficiency impaired VLCFA homeostasis and prolonged pro-inflammatory gene expression. Our study uncovers a pivotal role for ABCD1, a protein linked to neuroinflammation, and associated peroxisomal VLCFA degradation in regulating macrophage plasticity.

2022-11-09 | GSE217140 | GEO

Transcriptome of human primary macrophages from X-linked adrenoleukodystrophy patients and healthy controls before and after long-term myelin phagocytosis

Project description:Transcriptome of human primary macrophages from X-linked adrenoleukodystrophy patients and healthy controls before and after long-term myelin phagocytosis

| PRJNA1027446 | ENA

Gene expression profiles of fibroblasts from childhood cerebral adrenoleukodystrophy patients and healthy controls

2012-03-01 | GSE34308 | GEO

Gene expression profiles of fibroblasts from childhood cerebral adrenoleukodystrophy patients and healthy controls

Project description: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 Primary dermal fibroblast cultures from 5 CC-ALD patients and 5 healthy controls were cultured in DMEM medium supplemented with 10% FBS at 37°C with 5% CO2 until confluence for RNA extraction. The overall goal was to identify genes that are differentially expressed between CCALD patients and healthy controls

2012-03-01 | E-GEOD-34308 | biostudies-arrayexpress

Transcriptome of human primary macrophages from healthy controls after long-term myelin phagocytosis

Project description:Transcriptome of human primary macrophages from healthy controls after long-term myelin phagocytosis

| PRJNA1026622 | ENA

Gene expression profiles of fibroblasts and fibroblast-reprogrammed induced pluripotent stem cells (iPSCs) from childhood cerebral adrenoleukodystrophy patients and healthy controls

2012-03-01 | GSE34309 | GEO

Transcriptomic profile of macrophages activation state in healthy controls and MS patients

Project description:CD14+CD16- monocytes from MS patients and healthy controls (HC) were activated in vitro to obtain homeostatic-like, pro-inflammatory and pro-regenerative macrophages. Macrophage activation profiles were assessed through RNA sequencing

2024-10-29 | GSE269706 | GEO

Gene expression profiles of fibroblasts and fibroblast-reprogrammed induced pluripotent stem cells (iPSCs) from childhood cerebral adrenoleukodystrophy patients and healthy controls

Project description: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. We report the gene expression profiles of fibroblasts and fibroblast-reprogrammed iPSCs from childhood cerebral adrenoleukodystrophy patients and healthy controls Dermal fibroblast cultures from 2 CCALD patients and 3 healthy controls were reprogrammed into iPSCs by transfection with retroviruses desinged to express the human OCT4, SOX2, KLF4 and c-MYC cDNA. Fibroblasts and iPSCs were cultured in 1:1 ratio of DMEM/F12 medium supplemented with 20% KSR at 37°C with 5% CO2 until confluence for RNA extraction. The overall goal was to identify genes that are differentially expressed between CCALD patients and healthy controls.

2012-03-01 | E-GEOD-34309 | biostudies-arrayexpress

X-linked adrenoleukosdystrophy disease X-ALD

Project description:X-linked adrenoleukodystrophy (X-ALD) is a metabolic genetic disorder of the central nervous system characterized by axonopathy in spinal cords, progressive demyelination in the brain and adrenal insufficiency. Here we provide transcriptomic data from white matter of human X-ALD patients compared to healthy individuals. In particular, from two different disease variants: 1) patients with adrenomyeloneuropathy (AMN) with cerebral demyelination (cAMN) and, 2), an acute, ultimately lethal childhood cerebral form (cALD) featuring severe demyelination in the brain.

2012-03-21 | E-MEXP-3288 | biostudies-arrayexpress

Transcriptomic profile of macrophages activation state in healthy controls and MS patients

Project description:Transcriptomic profile of macrophages activation state in healthy controls and MS patients

| PRJNA1123271 | ENA