E-GEOD-48761 - Expression profiling of skin fibroblast and iPSC from Werner Syndrome

Status
Released on 11 July 2013, last updated on 24 July 2013
Organism
Homo sapiens
Samples (52)
Array (1)
Protocols (7)
Description
The premature aging disorder Werner Syndrome (WS) is characterized by early onset of aging phenotypes resembling natural aging. In most WS patients there are mutations in the DNA helicase WRN, an enzyme important in maintaining genome stability and telomere replication. Interestingly, its clinical manifestations reflect a severe degree of deterioration for connective tissue, whereas the central nervous system is less affected. We suggest that the varied vulnerability to aging is regulated by an unknown mechanism that protects specific lineages of stem cells from premature senescence. To address this problem, we reprogrammed patient skin fibroblasts to induced pluripotent stem cells (iPSC). The expression profile for the differentiated normal and WS fibroblasts and undifferentiated iPSC were compared. A distinct expression profile was found between normal and WS fibroblasts, however, few changes of gene expression were found in iPSC. Our findings suggest an erasure of aging phenotype associated with WS in reprogrammed iPSC. Human normal and WS skin fibroblasts were reprogrammed to induced pluripotent stem cells (iPSC). These samples, before and after reprogramming, were analyzed for the change of gene expression profile.
Experiment type
transcription profiling by array 
Contacts
Hoi Hung Cheung <cheungho@mail.nih.gov>, H H Cheung, L Thouennon, O M Rennert
MIAME
PlatformsProtocolsFactorsProcessedRaw
Files
Investigation descriptionE-GEOD-48761.idf.txt
Sample and data relationshipE-GEOD-48761.sdrf.txt
Raw data (2)E-GEOD-48761.raw.1.zip, E-GEOD-48761.raw.2.zip
Processed data (1)E-GEOD-48761.processed.1.zip
Array designA-AFFY-141.adf.txt
Links