Expression profiling suggests underexpression of the GABAA receptor subunit δ in the fragile X knockout mouse model

Ilse Gantois, Jo Vandesompele, Frank Speleman, Edwin Reyniers, Rudi D'Hooge, Lies Anne Severijnen, Rob Willemsen, Flora Tassone, R. Frank Kooy

Research output: Contribution to journalArticlepeer-review

125 Scopus citations


It is still unclear why absence of the fragile X protein (FMRP) leads to mental retardation and specific behavioral problems. In neurons, the protein transports specific mRNAs towards the actively translating ribosomes near the synapses. To unravel the mechanism leading to the disorder, we performed global gene expression analysis by means of the differential display method using the fragile X mouse model. To verify differential expression, we used microarray technology and real-time PCR. Three differentially expressed cDNAs showed consistent underexpression in the fragile X knockout mouse, including a GABAA receptor subunit δ, a Rho guanine exchange factor 12 and an EST BU563433. In addition, we identified 5 genes that showed differential expression dependent on the sample of RNA analysis. We consider their differential expression as provisional. It is possible that these differentially expressed genes play an important role in the cognitive and behavioral problems observed in the fragile X syndrome.

Original languageEnglish (US)
Pages (from-to)346-357
Number of pages12
JournalNeurobiology of Disease
Issue number2
StatePublished - Feb 2006


  • Fragile X knockout mouse
  • Fragile X syndrome
  • GABA receptor subunit δ
  • Rho guanine exchange factor 12

ASJC Scopus subject areas

  • Neurology


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