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E-GEOD-34308

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

OTHER RELATED OMICS DATASETS IN: PRJNA151513PRJNA151503

PROVIDER: E-GEOD-34308 | ExpressionAtlas | 2015-09-22

REPOSITORIES: ExpressionAtlas

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Homo sapiens

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

| PRJNA151513 | 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

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

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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

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

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E-GEOD-34309

2015-09-22 | E-GEOD-34309 | ExpressionAtlas

RNA-seq of induced brain microvascular endothelial cells from cerebral childhood adrenoleukodystrophy patients and wild-type controls

Project description:The goal of this study is to compare gene expression differences between induced brain microvascular endothelial cells from cerebral childhood adrenoleukodystrophy patients and wild-type controls

2018-04-01 | GSE108012 | GEO

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.

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2014-02-26 | E-GEOD-55319 | biostudies-arrayexpress

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