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| Status |
Public on Jul 13, 2012 |
| Title |
Expression data in induced pluripotent stem cells (iPSCs) derived from a DNA repair deficient fibroblast |
| Organism |
Homo sapiens |
| Experiment type |
Expression profiling by array
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| Summary |
Cockayne syndrome (CS) is an autossomal human disorder characterized by premature aging along with other symptoms. At the molecular level, CS is characterized by a deficiency in the Transcription-couple DNA repair pathway caused by a mutation mainly in ERCC6 gene and the absence of its functional protein. It has been shown that the presence of DNA damage and the lack of some functional proteins related to DNA repair constitute a barrier for somatic cell reprogramming. Recently, it was demonstrated that one protein involved in Genome Global Repair controls the expression of an important pluripotent gene, highligting its importance for cellular reprogramming.
We used microarray to confirm cellular reprogramming of CS fibroblasts at the molecular level and detail the expression of some genes invoved in cell death and aging control that might explain the unique characteristics of these iPSCs.
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| Overall design |
Normal (wild type) and fibroblasts from a CS patient were reprogrammed and independent clones were isolated. After sucessive passages in culture, total RNA from donor fibroblasts, iPSCs clones and human embryonic stem cell line (HUES6) were isolated and transcriptional analysis using human genome Affimetrix Gene Chip arrays were performed. HUES6 was used to demonstrate that CS and normal iPSCS show a global gene expression similar to an embryonic stem cell, confirming that both fibroblasts were successfully reprogrammed. We also used fibroblasts from CS to point out the diiferences in global gene expression after reprogramming. We sought to use normal (control) iPSCs to compare the levels of some genes involved in cell death and aging in iPSCs from CS fibroblasts.
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| Contributor(s) |
Muotri A, Yeo G |
| Citation(s) |
22661500 |
| Submission date |
Mar 20, 2012 |
| Last update date |
Jul 26, 2018 |
| Contact name |
Jason Nathanson |
| E-mail(s) |
nathanson@ucsd.edu
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| Organization name |
University of Californa San Diego
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| Department |
Cellular and Molecular Medicine
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| Lab |
Gene Yeo
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| Street address |
2880 Torrey Pines Rd, Rm 3807
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| City |
La Jolla |
| State/province |
CA |
| ZIP/Postal code |
92037 |
| Country |
USA |
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| Platforms (1) |
| GPL6244 |
[HuGene-1_0-st] Affymetrix Human Gene 1.0 ST Array [transcript (gene) version] |
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| Samples (24)
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| GSM898012 |
CSB-iPSC clone 1, replicate 1 |
| GSM898013 |
CSB-iPSC clone 1, replicate 2 |
| GSM898014 |
CSB-iPSC clone 1, replicate 3 |
| GSM898015 |
CSB-iPSC clone 2, replicate 1 |
| GSM898016 |
CSB-iPSC clone 2, replicate 2 |
| GSM898017 |
CSB-iPSC clone 2, replicate 3 |
| GSM898018 |
CSB-iPSC clone 3, replicate 1 |
| GSM898019 |
CSB-iPSC clone 3, replicate 2 |
| GSM898020 |
CSB-iPSC clone 3, replicate 3 |
| GSM898021 |
WT-iPSC clone 1, replicate 1 |
| GSM898022 |
WT-iPSC clone 1, replicate 2 |
| GSM898023 |
WT-iPSC clone 1, replicate 3 |
| GSM898024 |
WT-iPSC clone 2, replicate 1 |
| GSM898025 |
WT-iPSC clone 2, replicate 2 |
| GSM898026 |
WT-iPSC clone 2, replicate 3 |
| GSM898027 |
H1 replicate 1 |
| GSM898028 |
H1 replicate 2 |
| GSM898029 |
H1 replicate 3 |
| GSM898030 |
HUES6 replicate 1 |
| GSM898031 |
HUES6 replicate 2 |
| GSM898032 |
HUES6 replicate 3 |
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| Relations |
| BioProject |
PRJNA153655 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE36648_RAW.tar |
88.8 Mb |
(http)(custom) |
TAR (of CEL) |
| Processed data included within Sample table |
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