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Series GSE48761 Query DataSets for GSE48761
Status Public on Jul 11, 2013
Title Expression profiling of skin fibroblast and iPSC from Werner Syndrome
Organism Homo sapiens
Experiment type Expression profiling by array
Summary 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.
 
Overall design 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.
 
Contributor(s) Cheung HH, Thouennon L, Rennert OM
Citation(s) 24749076
Submission date Jul 10, 2013
Last update date Jul 26, 2018
Contact name Hoi Hung Cheung
E-mail(s) cheungho@mail.nih.gov
Phone 301-451-8368
Organization name NICHD
Lab Laboratory of Clinical and Developmental Genomics
Street address 49 Convent Dr, Rm.2C08 Bldg.49
City Bethesda
State/province MD
ZIP/Postal code 20892
Country USA
 
Platforms (1)
GPL6244 [HuGene-1_0-st] Affymetrix Human Gene 1.0 ST Array [transcript (gene) version]
Samples (52)
GSM1184254 Normal Fibroblast AN1 rep1
GSM1184255 Normal Fibroblast AN1 rep2
GSM1184256 Normal Fibroblast AN2 rep1
Relations
BioProject PRJNA211411

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Supplementary file Size Download File type/resource
GSE48761_RAW.tar 211.2 Mb (http)(custom) TAR (of CEL)
Processed data included within Sample table

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