title: Expression profiling suggests underexpression of the GABA(A) receptor subunit delta in the fragile X knockout mouse model.
abstract: 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 GABA(A) receptor subunit delta, 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.
authors.affiliation: Department of Medical Genetics, University of Antwerp, Universiteitsplein 1, 2610 Antwerp, Belgium.
authors.forename: Ilse
authors.initials: I
authors.lastname: Gantois
authors.forename: Jo
authors.initials: J
authors.lastname: Vandesompele
authors.forename: Frank
authors.initials: F
authors.lastname: Speleman
authors.forename: Edwin
authors.initials: E
authors.lastname: Reyniers
authors.forename: Rudi
authors.initials: R
authors.lastname: D'Hooge
authors.forename: Lies-Anne
authors.initials: LA
authors.lastname: Severijnen
authors.forename: Rob
authors.initials: R
authors.lastname: Willemsen
authors.forename: Flora
authors.initials: F
authors.lastname: Tassone
authors.forename: R Frank
authors.initials: RF
authors.lastname: Kooy
mesh_terms: D000818:Animals; D001921:Brain / Q000502:physiology*; D004195:Disease Models, Animal; D051860:Fragile X Messenger Ribonucleoprotein 1 / Q000235:genetics; D005600:Fragile X Syndrome / Q000235:genetics*; D020869:Gene Expression Profiling; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D020411:Oligonucleotide Array Sequence Analysis; D018079:Receptors, GABA / Q000378:metabolism*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012689:Sequence Homology, Nucleic Acid
chemical_list: D051860:Fragile X Messenger Ribonucleoprotein 1; D018079:Receptors, GABA