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Series GSE34309 Query DataSets for GSE34309
Status Public on Mar 01, 2012
Title Gene expression profiles of fibroblasts and fibroblast-reprogrammed induced pluripotent stem cells (iPSCs) from childhood cerebral adrenoleukodystrophy patients and healthy controls
Organism Homo sapiens
Experiment type Expression profiling by array
Summary 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
Overall design 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.
Contributor(s) Wang X, Hacia JG
Citation(s) 23036268
Submission date Dec 09, 2011
Last update date Dec 06, 2018
Contact name Joseph Gerard Hacia
Phone 323-442-3030
Organization name University of Southern California
Department Biochemistry and Molecular Biology
Lab Hacia Lab
Street address 2250 Alcazar Street, IGM 261
City Los Angeles
State/province CA
ZIP/Postal code 90089
Country USA
Platforms (1)
GPL571 [HG-U133A_2] Affymetrix Human Genome U133A 2.0 Array
Samples (18)
GSM847088 healthy control fibroblast, biological rep1
GSM847089 healthy control fibroblast, biological rep2
GSM847090 healthy control fibroblast, biological rep3
BioProject PRJNA151503

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Supplementary file Size Download File type/resource
GSE34309_RAW.tar 34.7 Mb (http)(custom) TAR (of CEL)
Processed data included within Sample table

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