Transcriptomics

Dataset Information

4

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


ABSTRACT: 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

ORGANISM(S): Homo sapiens  

SUBMITTER: Tom Burdon   Jan-Willem Taanman  Fatima Cavaleri  Tilo Kunath  Masumi Nagano  Henry Houlden  Katrina Gwinn  Patrick A Lewis  Alison J Thomson  Petr Vodicka  John Hardy  Nicola J Drummond  Mina Ryten  Michael J Devine  Anthony H Schapira 

PROVIDER: E-GEOD-30792 | ArrayExpress | 2011-09-14

SECONDARY ACCESSION(S): GSE30792PRJNA144289

REPOSITORIES: GEO, ArrayExpress

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A major barrier to research on Parkinson's disease is inaccessibility of diseased tissue for study. One solution is to derive induced pluripotent stem cells from patients and differentiate them into neurons affected by disease. Triplication of SNCA, encoding α-synuclein, causes a fully penetrant, aggressive form of Parkinson's disease with dementia. α-Synuclein dysfunction is the critical pathogenic event in Parkinson's disease, multiple system atrophy and dementia with Lewy bodies. Here we prod  ...[more]

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