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| 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
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| 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
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| 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.
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| Contributor(s) |
Wang X, Hacia JG |
| Citation(s) |
23036268 |
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| Submission date |
Dec 09, 2011 |
| Last update date |
Dec 06, 2018 |
| Contact name |
Joseph Gerard Hacia |
| E-mail(s) |
hacia@hsc.usc.edu
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| Phone |
323-442-3030
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| Organization name |
University of Southern California
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| Department |
Biochemistry and Molecular Biology
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| Lab |
Hacia Lab
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| Street address |
2250 Alcazar Street, IGM 261
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| City |
Los Angeles |
| State/province |
CA |
| ZIP/Postal code |
90089 |
| Country |
USA |
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| Platforms (1) |
| GPL571 |
[HG-U133A_2] Affymetrix Human Genome U133A 2.0 Array |
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| Samples (18)
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| GSM847088 |
healthy control fibroblast, biological rep1 |
| GSM847089 |
healthy control fibroblast, biological rep2 |
| GSM847090 |
healthy control fibroblast, biological rep3 |
| GSM847091 |
CCALD patient fibroblast, biological rep1 |
| GSM847092 |
CCALD patient fibroblast, biological rep2 |
| GSM847093 |
iPSC derived from healthy control fibroblast WT1, biological rep1 |
| GSM847094 |
iPSC derived from healthy control fibroblast WT1, biological rep2 |
| GSM847095 |
iPSC derived from healthy control fibroblast WT1, biological rep3 |
| GSM847096 |
iPSC derived from healthy control fibroblast WT2, biological rep4 |
| GSM847097 |
iPSC derived from healthy control fibroblast WT2, biological rep5 |
| GSM847098 |
iPSC derived from healthy control fibroblast WT2, biological rep6 |
| GSM847099 |
iPSC derived from healthy control fibroblast WT2, biological rep7 |
| GSM847100 |
iPSC derived from healthy control fibroblast WT3, biological rep8 |
| GSM847101 |
iPSC derived from CCALD patient fibroblast CCALD1, biological rep1 |
| GSM847102 |
iPSC derived from CCALD patient fibroblast CCALD1, biological rep2 |
| GSM847103 |
iPSC derived from CCALD patient fibroblast CCALD1, biological rep3 |
| GSM847104 |
iPSC derived from CCALD patient fibroblast CCALD1, biological rep4 |
| GSM847105 |
iPSC derived from CCALD patient fibroblast CCALD2, biological rep5 |
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| Relations |
| BioProject |
PRJNA151503 |
| 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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