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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

DISEASE(S): X-linked Cerebral Adrenoleukodystrophy,Normal

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

REPOSITORIES: ExpressionAtlas

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			Action	DRS
	E-GEOD-34308-analysis-methods.tsv	Tabular
	E-GEOD-34308-atlasExperimentSummary.Rdata	Rdata
	E-GEOD-34308-configuration.xml	Xml
	E-GEOD-34308-percentile-ranks.tsv	Tabular
	E-GEOD-34308.condensed-sdrf.tsv	Tabular

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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

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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.

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