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

被引：127

作者：

Gantois, I

Vandesompele, J

Speleman, F

Reyniers, E

D'Hooge, R

Severijnen, LA

Willemsen, R

Tassone, F

Kooy, RF

机构：

[1] Univ Antwerp, Dept Med Genet, B-2610 Antwerp, Belgium

[2] Ghent Univ Hosp, Dept Med Genet, Ghent, Belgium

[3] Univ Antwerp, Born Bunge Fdn, Dept Neurochem & Behav, B-2020 Antwerp, Belgium

[4] Erasmus MC, Dept Clin Genet, Rotterdam, Netherlands

[5] Univ Calif Davis, Sch Med, Dept Biol Chem, Sacramento, CA 95817 USA

来源：

NEUROBIOLOGY OF DISEASE | 2006年 / 21卷 / 02期

关键词：

fragile X syndrome; fragile X knockout mouse; GABA(A); receptor subunit delta; Rho guanine exchange factor 12;

D O I：

10.1016/j.nbd.2005.07.017

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

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 6, 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. 0 2005 Elsevier Inc. All rights reserved.

引用

页码：346 / 357

页数：12

共 85 条

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