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 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 language | English (US) |
|---|---|
| Pages (from-to) | 346-357 |
| Number of pages | 12 |
| Journal | Neurobiology of Disease |
| Volume | 21 |
| Issue number | 2 |
| DOIs | |
| State | Published - Feb 2006 |
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Keywords
- Fragile X knockout mouse
- Fragile X syndrome
- GABA receptor subunit δ
- Rho guanine exchange factor 12
ASJC Scopus subject areas
- Neurology
Cite this
Expression profiling suggests underexpression of the GABAA receptor subunit δ in the fragile X knockout mouse model. / Gantois, Ilse; Vandesompele, Jo; Speleman, Frank; Reyniers, Edwin; D'Hooge, Rudi; Severijnen, Lies Anne; Willemsen, Rob; Tassone, Flora; Kooy, R. Frank.
In: Neurobiology of Disease, Vol. 21, No. 2, 02.2006, p. 346-357.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Expression profiling suggests underexpression of the GABAA receptor subunit δ in the fragile X knockout mouse model
AU - Gantois, Ilse
AU - Vandesompele, Jo
AU - Speleman, Frank
AU - Reyniers, Edwin
AU - D'Hooge, Rudi
AU - Severijnen, Lies Anne
AU - Willemsen, Rob
AU - Tassone, Flora
AU - Kooy, R. Frank
PY - 2006/2
Y1 - 2006/2
N2 - 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.
AB - 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.
KW - Fragile X knockout mouse
KW - Fragile X syndrome
KW - GABA receptor subunit δ
KW - Rho guanine exchange factor 12
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UR - http://www.scopus.com/inward/citedby.url?scp=30744433092&partnerID=8YFLogxK
U2 - 10.1016/j.nbd.2005.07.017
DO - 10.1016/j.nbd.2005.07.017
M3 - Article
C2 - 16199166
AN - SCOPUS:30744433092
VL - 21
SP - 346
EP - 357
JO - Neurobiology of Disease
JF - Neurobiology of Disease
SN - 0969-9961
IS - 2
ER -