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Series GSE36648 Query DataSets for GSE36648
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
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.
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.
Contributor(s) Muotri A, Yeo G
Citation(s) 22661500
Submission date Mar 20, 2012
Last update date Jul 26, 2018
Contact name Jason Nathanson
Organization name University of Californa San Diego
Department Cellular and Molecular Medicine
Lab Gene Yeo
Street address 2880 Torrey Pines Rd, Rm 3807
City La Jolla
State/province CA
ZIP/Postal code 92037
Country USA
Platforms (1)
GPL6244 [HuGene-1_0-st] Affymetrix Human Gene 1.0 ST Array [transcript (gene) version]
Samples (24)
GSM898009 CSB-FB replicate 1
GSM898010 CSB-FB replicate 2
GSM898011 CSB-FB replicate 3
BioProject PRJNA153655

Download family Format
SOFT formatted family file(s) SOFTHelp
MINiML formatted family file(s) MINiMLHelp
Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE36648_RAW.tar 88.8 Mb (http)(custom) TAR (of CEL)
Raw data provided as supplementary file
Processed data included within Sample table

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