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Series GSE40378 Query DataSets for GSE40378
Status Public on Oct 25, 2012
Title Induced pluripotent stem cell models of progranulin-deficient frontotemporal dementia uncover specific reversible neuronal defects.
Organism Homo sapiens
Experiment type Expression profiling by array
Summary Multiple FTD patient-specific iPSC lines were generated for the first time, Human neurons of progranulin haploinsufficiency have been established.
PGRN S116X neurons are more sensitive to kinase inhibitors-induced cell stress, which can be rescued by ectopic progranulin expression, revealing progranulin-dependent cellular defects in FTD. Microarray analysis reveals that the serine/threonine kinase S6K2 (RPS6KB2) and other genes involved in MAPK signaling are dysregulated specifically in neurons with progranulin deficiency.
 
Overall design 32 independent human neuronal cultures were analyzed in this study
 
Contributor(s) Almeida S, Zhang Z, Coppola G, Mao W, Futai K, Karydas A, Geschwind MD, Tartaglia MC, Gao F, Gianni D, Sena-Esteves M, Geschwind DH, Miller BL, Farese RV Jr, Gao F
Citation(s) 23063362
Submission date Aug 24, 2012
Last update date Aug 13, 2018
Contact name Giovanni Coppola
E-mail gcoppola@ucla.edu
Phone 310-794-4172
Organization name UCLA
Department Psychiatry and Neurology
Lab Neurogenetics
Street address 1524 Gonda, 695 Charles Young Drive South
City Los Angeles
State/province CA
ZIP/Postal code 90095
Country USA
 
Platforms (1)
GPL10558 Illumina HumanHT-12 V4.0 expression beadchip
Samples (32)
GSM992522 N_FTD1_U29
GSM992523 N_CTR2_U10
GSM992524 N_FTD2_U13
Relations
BioProject PRJNA173801

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
GSE40378_RAW.tar 26.2 Mb (http)(custom) TAR
GSE40378_non_normalized.txt.gz 5.8 Mb (ftp)(http) TXT
Raw data is available on Series record
Processed data included within Sample table

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