Transcriptomics

Dataset Information

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Global gene expression analysis of human iPSC-derived Neural Stem Cells (NSC) from LCA patients and unaffected persons


ABSTRACT: Our purpose was to investigate genes and molecular mechanisms involved in patients with Leber congenital amaurosis (LCA). Fibroblasts from two unrelated clinically-identified patients (Coriell) were reprogrammed to pluripotency by retroviral transduction. These human induced Pluripotent Stem Cells (hiPSCs) were differentiated into neural stem cells (NSC) that mimicked the neural tube stage and retinal pigmented epithelial (RPE) cells that could be targeted by the disease. A genome wide transcriptome analysis was performed with Affymetrix Exon Array GeneChip®, comparing LCA-hiPSCs derivatives to controls. The aim was to identify differentially expressed genes which may be associated with early developmental defect before the establishment of mature retinal circuitry. We analyzed iPSC-derived neural stem cells from LCA patient's fibroblast (n=2) and iPSC-derived neural stem cells from healthy people fibroblast (n=2). A total of 21 samples were analyzed : 9 NSC derived from iPSC LCA and 12 NSC derived from wild-type iPSC.

ORGANISM(S): Homo sapiens  

SUBMITTER: Christelle Monville  Celine Lustremant   Pierre de la Grange    

PROVIDER: E-GEOD-43924 | ArrayExpress | 2015-01-27

SECONDARY ACCESSION(S): GSE43924PRJNA188200

REPOSITORIES: GEO, ArrayExpress

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Publications

Human induced pluripotent stem cells as a tool to model a form of Leber congenital amaurosis.

Lustremant Céline C   Habeler Walter W   Plancheron Alexandra A   Goureau Olivier O   Grenot Lydie L   de la Grange Pierre P   Audo Isabelle I   Nandrot Emeline F EF   Monville Christelle C  

Cellular reprogramming 20130510 3


Our purpose was to investigate genes and molecular mechanisms involved in patients with Leber congenital amaurosis (LCA) and to model this type of LCA for drug screening. Fibroblasts from two unrelated clinically identified patients with a yet undetermined gene mutation were reprogrammed to pluripotency by retroviral transduction. These human induced pluripotent stem cells (hiPSCs) were differentiated into neural stem cells (NSCs) that mimicked the neural tube stage and retinal pigmented epithel  ...[more]

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