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

Neurobiology of Disease (Feb 2006)

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

Affiliations

Ilse Gantois: Department of Medical Genetics, University of Antwerp, Universiteitsplein 1, 2610 Antwerp, Belgium
Jo Vandesompele: Department of Medical Genetics, Ghent University Hospital, Ghent, Belgium
Frank Speleman: Department of Medical Genetics, Ghent University Hospital, Ghent, Belgium
Edwin Reyniers: Department of Medical Genetics, University of Antwerp, Universiteitsplein 1, 2610 Antwerp, Belgium
Rudi D'Hooge: Department of Neurochemistry and Behavior, Born-Bunge Foundation, University of Antwerp, Antwerp, Belgium
Lies-Anne Severijnen: Department of Clinical Genetics, Erasmus MC, Rotterdam, The Netherlands
Rob Willemsen: Department of Clinical Genetics, Erasmus MC, Rotterdam, The Netherlands
Flora Tassone: Department of Biological Chemistry, University of California, Davis, School of Medicine, Davis, CA 95817, USA
R. Frank Kooy: Department of Medical Genetics, University of Antwerp, Universiteitsplein 1, 2610 Antwerp, Belgium; Corresponding author. Fax: +32 3 820 25 66.

Journal volume & issue: Vol. 21, no. 2
pp. 346 – 357

Abstract

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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.

Published in Neurobiology of Disease

ISSN: 0969-9961 (Print); 1095-953X (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Neurosciences. Biological psychiatry. Neuropsychiatry
Website: https://www.journals.elsevier.com/neurobiology-of-disease

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