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Mice Lacking the 3 Subunit of the GABAA Receptor Have the Epilepsy Phenotype and Many of the Behavioral Characteristics of
 

Summary: Mice Lacking the 3 Subunit of the GABAA Receptor Have the
Epilepsy Phenotype and Many of the Behavioral Characteristics of
Angelman Syndrome
T. M. DeLorey,1 A. Handforth,3 S. G. Anagnostaras,2 G. E. Homanics,4 B. A. Minassian,3 A. Asatourian,3
M. S. Fanselow,2 A. Delgado-Escueta,2 G. D. Ellison,2 and R. W. Olsen1
Departments of 1Molecular and Medical Pharmacology and 2Psychology, University of California, Los Angeles, California
90095, and 3Neurology Division, West Los Angeles Veteran's Affairs Medical Center, Los Angeles, California 90073, and
4Department of Anesthesiology/Critical Care Medicine, University of Pittsburgh, Pennsylvania 15261
Angelman syndrome (AS) is a severe neurodevelopmental dis-
order resulting from a deletion/mutation in maternal chromo-
some 15q1113. The genes in 15q1113 contributing to the full
array of the clinical phenotype are not fully identified. This study
examines whether a loss or reduction in the GABAA receptor 3
subunit (GABRB3) gene, contained within the AS deletion re-
gion, may contribute to the overall severity of AS. Disrupting the
gabrb3 gene in mice produces electroencephalographic abnor-
malities, seizures, and behavior that parallel those seen in AS.
The seizures that are observed in these mice showed a phar-
macological response profile to antiepileptic medications sim-
ilar to that observed in AS. Additionally, these mice exhibited

  

Source: Anagnostaras, Stephan - Neurosciences Program & Department of Psychology, University of California at San Diego

 

Collections: Biology and Medicine