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E-GEOD-30792 - Expression data from Parkison's iPSCs with four copies of SNCA, and equivalent cell lines from an unaffected first degree relative

Status
Released on 14 September 2011, last updated on 29 September 2011
Organism
Homo sapiens
Samples (5)
Array (1)
Protocols (7)
Description
A major barrier to research on Parkinson’s disease (PD) is inaccessibility of diseased tissue for study. One solution is to derive induced pluripotent stem cells (iPSCs) from patients with PD and differentiate them into neurons affected by disease. We created an iPSC model of PD caused by triplication of SNCA encoding α-synuclein. α-Synuclein dysfunction is common to all forms of PD, and SNCA triplication leads to fully penetrant familial PD with accelerated pathogenesis. After differentiation of iPSCs into neurons enriched for midbrain dopaminergic subtypes, those from the patient contain double α-synuclein protein compared to those from an unaffected relative, precisely recapitulating the cause of PD in these individuals. A measurable biomarker makes this model ideal for drug screening for compounds that reduce levels of α-synuclein, and for mechanistic experiments to study PD pathogenesis. This gene expression microarray study was carried out as part of the validation process for demonstrating that the generated iPSC lines are pluripotent. 5 samples were analysed: two clonal iPSC lines from each of two genotypes (four in total; AST denoting alpha-synuclein triplication and NAS denoting normal alpha-synuclein), a human embryonic stem cell line (SHEF4). All cultured in self-renewal conditions, mTeSR1
Experiment type
transcription profiling by array 
Contacts
Alison J Thomson, Anthony H Schapira, Fatima Cavaleri, Henry Houlden, Jan-Willem Taanman, John Hardy, Katrina Gwinn, Masumi Nagano, Michael J Devine, Mina Ryten, Nicola J Drummond, Patrick A Lewis, Petr Vodicka, Tilo Kunath, Tom Burdon
Citation
Parkinson's disease induced pluripotent stem cells with triplication of the α-synuclein locus. Devine MJ, Ryten M, Vodicka P, Thomson AJ, Burdon T, Houlden H, Cavaleri F, Nagano M, Drummond NJ, Taanman JW, Schapira AH, Gwinn K, Hardy J, Lewis PA, Kunath T. , PMID:21863007
MIAME
PlatformsProtocolsVariablesProcessedRaw
Files
Investigation descriptionE-GEOD-30792.idf.txt
Sample and data relationshipE-GEOD-30792.sdrf.txt
Raw data (1)E-GEOD-30792.raw.1.zip
Processed data (1)E-GEOD-30792.processed.1.zip
Array designA-AFFY-44.adf.txt
R ExpressionSetE-GEOD-30792.eSet.r
Links