Expression in Superior Frontal Gyrus of normal individuals or females of different ages affected by Rett syndrome

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PubMed ID: 17309881
Contact: valerie matagne

Image:email_GSE6955.png
OHSU

Species: Homo sapiens
Platform: Affymetrix GeneChip Human Genome U95 Version [1 or 2] Set HG-U95A
Number of Samples: 6
Date Range: 08/30/2001 - 12/17/2001
Experiment Quality: 49.47
9.43 %
70.02
Link to Repository: GSE6955
Original Intensity Data: Download
Clean Intensity Data: Download
Original PLIER Data: Download
Clean PLIER Data: Download
Clean CEL Files: Download
Data Rank:

Rett syndrome (RTT, OMIM #312750) is a severe X-linked neurodevelopmental disorder linked to heterozygous de novo mutations in the MECP2 gene. MECP2 encodes methyl-CpG-binding protein 2 (MeCP2), which represses gene transcription by binding to 5-methylcytosine residues in symmetrically positioned CpG dinucleotides. The disorder is almost exclusively diagnosed in females, because males affected by the disease usually die perinatally due to severe encephalopathy. Direct MeCP2 target genes underlying the neuropathogenesis of RTT remain largely unknown.

To identify genes involved in the neuropathogenesis of RTT we assessed the gene expression profile of postmortem brains affected by RTT and that of age-matched normal brains using Affymetrix arrays.

Design: samples of the Superior Frontal Gyrus from normal and RTT patients of different ages were obtained from the Krieger Research Institute and the Harvard Brain Tissue Ressource Center. Samples from RTT-affected girls (2-4, 6 and 8 year-old) were compared with aged-matched normal brain tissue (2, 5 and 10 year-old). To ensure the quality of hybridization , labeled RNA was hybridized to a Test Array containing probe sets for all Affymetrix Expression Array controls. Background, noise, average Avg Diff, and ratio of Avg Diff values for probe sets representing the 5’ and 3’ ends of actin and GAPDH transcripts were used to determine target quality. Targets that did not meet empirically determined cut-off values within the project were remade. Quality tested samples were then hybridized to the HU_U95Av2 array. Quality-tested samples were then hybridized in the HU_U95Av2 array containing 12,625 human probes.

Type: comparison between Rettsyndrome suffering and non-suffering patients


Contents

[edit] Contributors

  • Vivianne Deng
  • Valerie Matagne
  • Sergio R Ojeda

[edit] Protocols

[edit] References

[edit] Links

GEO Accession for this experiment: GSE6955. (Public on Feb 05 2007)

PubMed abstract: 17309881

RAW data: tar file.

[edit] Analysis

[edit] Samples/Quality Control

Sample Date Scanned Source Link Probe Variance Data Artifact Mask Quality Score
Superior Frontal Gyrus_2y_normal 12/17/01 14:44:23 GSM160306 Probe variance for  Superior Frontal Gyrus_2y_normal Artifact mask for  Superior Frontal Gyrus_2y_normal 69.6421
0.22 %
84.71
Superior Frontal Gyrus_2-4y_RTT 12/17/01 14:18:31 GSM160307 Probe variance for  Superior Frontal Gyrus_2-4y_RTT Artifact mask for  Superior Frontal Gyrus_2-4y_RTT 70.3066
0.07 %
85.12
Superior Frontal Gyrus_5y_normal 11/01/01 11:01:11 GSM160308 Probe variance for  Superior Frontal Gyrus_5y_normal Artifact mask for  Superior Frontal Gyrus_5y_normal 68.6204
1.52 %
83.55
Brain area 8/9_6y_RTT 08/30/01 13:00:29 GSM160309 Probe variance for  Brain area 8/9_6y_RTT Artifact mask for  Brain area 8/9_6y_RTT 69.4022
0.23 %
84.59
Superior Frontal Gyrus_10y_normal 12/17/01 14:54:42 GSM160310 Probe variance for  Superior Frontal Gyrus_10y_normal Artifact mask for  Superior Frontal Gyrus_10y_normal -0.016039
63.88 %
18.0496
Brain area 8/9_8yp_RTT 12/17/01 14:28:57 GSM160311 Probe variance for  Brain area 8/9_8yp_RTT Artifact mask for  Brain area 8/9_8yp_RTT 69.908
0.01 %
84.9256
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