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The expression patterns correlated with disease severity of adrenoleukodystrophy via microarray analysis

ABSTRACT: To gain molecular insight into the inflammatory phenotype of childhood cerebral adrenoleukodystrophy (CCALD), we performed microarray-based transcriptional profiling analysis of human embryonic stem cells (hESCs), control-induced pluripotent stem cells (iPSCs), adrenomyeloneuropathy (AMN)-iPSCs and CCALD-iPSCs.

ORGANISM(S): Homo sapiens

PROVIDER: GSE85804 | GEO | 2016/08/19

SECONDARY ACCESSION(S): PRJNA339431

REPOSITORIES: GEO

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The expression patterns correlated with disease severity of adrenoleukodystrophy via microarray analysis

Project description:To gain molecular insight into the inflammatory phenotype of childhood cerebral adrenoleukodystrophy (CCALD), we performed microarray-based transcriptional profiling analysis of human embryonic stem cells (hESCs), control-induced pluripotent stem cells (iPSCs), adrenomyeloneuropathy (AMN)-iPSCs and CCALD-iPSCs. 200 ng of total RNA from purified hESCs (P35â45), control-iPSCs (P10-20), CCALD-iPSCs (P10â20), AMN-iPSCs (P10â20) was transcribed to yield biotinylated complimentary RNA (cRNA) according to the manufacturerâs instructions.

2016-08-19 | E-GEOD-85804 | biostudies-arrayexpress

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Project description:The Transcriptomic analysis of RNA samples from adrenomyeloneuropathy (AMN) and childhood cerebral adrenoleukodystrophy (CCALD), and healthy control transcriptomics data was conducted to elucidate distinctive metabolic biosignatures among AMN and CCALD, and healthy control

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

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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

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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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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

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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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