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| Status |
Public on Jul 24, 2010 |
| Title |
Gene expression profiling of neural stem cells derived from iPS cells (iPSc) of Sanfilippo syndrome type B (MPSIIIB) patient versus control |
| Organism |
Homo sapiens |
| Experiment type |
Expression profiling by array
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| Summary |
We generated iPSc from skin fibroblasts of two MPSIIIB patients (P1 and P2). MPSIIIB-associated cell defects were prominent in undifferentiated iPSc, in neural stem cells and in their neuronal progeny.
We explored alterations of metabolic pathways in MPSIIIB neural cells by performing gene expression profiling of patient versus control neural stem cells. Exon array transcriptome analysis showed 295 transcripts with increased expression level and 1275 transcripts with decreased expression level in patient versus control neural precursors.
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| Overall design |
Total RNA was extracted from proliferating neurosphere cultures derived from two control (C1 and C3) and three patient (P1.1, P1.3, and P2.3) iPSc clones. We considered a minimal fold change of 1.5 fold and a corrected P value lower than 0.05.
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| Contributor(s) |
Bohl D, Lemonnier T, Blanchard S, Heard JM |
| Citation(s) |
21685203 |
| Submission date |
Jul 22, 2010 |
| Last update date |
Feb 18, 2019 |
| Contact name |
Pierre de la Grange |
| E-mail(s) |
pierre.delagrange@genosplice.com
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| Organization name |
GenoSplice technology
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| Department |
Paris Biotech Santé
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| Street address |
29, rue du Faubourg Saint-Jacques
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| City |
Paris |
| ZIP/Postal code |
75014 |
| Country |
France |
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| Platforms (1) |
| GPL5175 |
[HuEx-1_0-st] Affymetrix Human Exon 1.0 ST Array [transcript (gene) version] |
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| Samples (5)
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| Relations |
| BioProject |
PRJNA131683 |
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