Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

Dataset Information

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Transcription profiling of mouse E8.5 wild-type and 3 E8.5 Tcof1+/- littermate embryos (Treacher Collins Syndrome)


ABSTRACT: The object of this study was to identify genes transcriptionally upregulated and downregulated in response to Tcof1 haploin-sufficiency during mouse embryogensis Experiment Overall Design: Total RNA was extracted from 3 E8.5 wild-type and 3 E8.5 Tcof1+/- littermate embryos using the RNeasy Mini Protocol for Isolation of Total RNA from Animal Tissues (Qiagen) according to the manufacturer’s protocol. RNA quality and quantity was determined using the Bioanalyser. To generate targets for microarray analysis, total RNA (100 ng) from each wild-type and mutant embryo was amplified using Two-Cycle Target Labeling (Affymetrix) according to the manufacturer’s instructions. Biotinylated target cRNAs (20 μg) from the 3 wild-type and 3 Tcof1+/- embryos were hybridised to separate GeneChip® Mouse Genome 430 2.0 arrays (Affymetrix), following standard Affymetrix procedures.

ORGANISM(S): Mus musculus

SUBMITTER: Gaye Hattem 

PROVIDER: E-GEOD-10167 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

Prevention of the neurocristopathy Treacher Collins syndrome through inhibition of p53 function.

Jones Natalie C NC   Lynn Megan L ML   Gaudenz Karin K   Sakai Daisuke D   Aoto Kazushi K   Rey Jean-Phillipe JP   Glynn Earl F EF   Ellington Lacey L   Du Chunying C   Dixon Jill J   Dixon Michael J MJ   Trainor Paul A PA  

Nature medicine 20080203 2


Treacher Collins syndrome (TCS) is a congenital disorder of craniofacial development arising from mutations in TCOF1, which encodes the nucleolar phosphoprotein Treacle. Haploinsufficiency of Tcof1 perturbs mature ribosome biogenesis, resulting in stabilization of p53 and the cyclin G1-mediated cell-cycle arrest that underpins the specificity of neuroepithelial apoptosis and neural crest cell hypoplasia characteristic of TCS. Here we show that inhibition of p53 prevents cyclin G1-driven apoptoti  ...[more]

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