Audiogenic Seizures Susceptibility in Transgenic Mice with Fragile X Syndrome
Top Cited Papers
- 1 December 2000
- Vol. 41 (1), 19-23
- https://doi.org/10.1111/j.1528-1157.2000.tb01499.x
Abstract
To evaluate their susceptibility to audiogenic seizures, five groups of knockout mice with various forms of fragile X genetic involvement [hemizygous males (n = 46), and homozygous (n = 38) and heterozygous females (n = 45), and their normal male (n = 45) and female (n = 52) littermates] were studied. All mouse groups were tested at ages 17, 22, 35, and 45 days. Audiogenic seizure susceptibility was scored, and the analysis of variance was used for the evaluation of the effects of age and genetic condition on seizure severity score (SSS). All groups of knockout fragile X mice exhibited SSSs significantly higher than those observed in their wild-type littermates; among knockout mice, hemizygous males and homozygous females showed the highest SSSs. Hemizygous males showed higher SSSs with increasing age, from 17 to 45 days; homozygous females showed a peak at age 22 days, followed by a decrease; finally, heterozygous females had their highest SSSs at age 17 days. This study demonstrates that an increased susceptibility to audiogenic seizures is present in fragile X knockout mice at all the ages tested. These results support the validity of this animal model also for epilepsy and seizures in the human fragile X syndrome.This publication has 28 references indexed in Scilit:
- Epilepsy and EEG Findings in Males with Fragile X SyndromeEpilepsia, 1999
- Sound-induced seizures in serotonin 5-HT2c receptor mutant miceNature Genetics, 1997
- Transgenic mouse model for the fragile X syndromeAmerican Journal of Medical Genetics, 1996
- Conservation of CGG region in FMR1 gene in mammalsAmerican Journal of Medical Genetics, 1994
- Human and murine FMR-1: alternative splicing and translational initiation downstream of the CGG–repeatNature Genetics, 1993
- Characterization and localization of the FMR-1 gene product associated with fragile X syndromeNature, 1993
- Variation of the CGG repeat at the fragile X site results in genetic instability: Resolution of the Sherman paradoxCell, 1991
- Absence of expression of the FMR-1 gene in fragile X syndromeCell, 1991
- Mapping of two genes that influence susceptibility to audiogenic seizures in crosses of C57BL/6J and DBA/2J miceBehavior Genetics, 1990
- Fragile‐X Syndrome: A Particular Epileptogenic EEG PatternEpilepsia, 1988