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The Journal of Neuroscience, March 30, 2005, 25(13):3452-3458; doi:10.1523/JNEUROSCI.0231-05.2005

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Neurobiology of Disease
Development of a New Genetic Model for Absence Epilepsy: Spike-Wave Seizures in C3H/He and Backcross Mice

Wayne N. Frankel,¹ Barbara Beyer,¹ Christina R. Maxwell,² Stephanie Pretel,¹ Verity A. Letts,¹ and Steven J. Siegel²

¹The Jackson Laboratory, Bar Harbor, Maine 04609, and ²Division of Neuropsychiatry, University of Pennsylvania, Philadelphia, Pennsylvania 19104

To characterize the genetic basis of spike-wave discharges (SWDs) detected by electroencephalography (EEG) in C3H/He mice, substrains of C3H mice were evaluated by EEG and sensitivity to ethosuximide. Crosses with the SWD-negative strain C57BL/6J were performed to map the underlying gene(s). C3H/He substrains exhibited a modest incidence (average of 19 SWDs per hour) of 7-8 Hz SWDs when at rest, compared with the C3HeB/Fe subline (four SWDs per hour). In the mapping backcross, however, many mice showed a very high incidence (50-220 SWDs per hour) throughout the recording period. SWDs were first detected at 3.5 weeks of age, were associated with behavioral arrest, were suppressed by ethosuximide, and were strongest in the cerebral cortex and thalamus. The major C3H determinant of SWDs, spkw1 (spike-wave 1), mapped to chromosome (Chr 9), and together with a C57BL/6J determinant on Chr 8, spkw2, accounted for more than one-half of the phenotypic variation in the backcross mice. The modest SWD incidence in C3H/He mice and the high incidence in backcrosses implies that SWD could be a confounding variable for other behaviors. Because C3H/He mice have no other brain abnormalities, they are an attractive alternative for studying idiopathic absence epilepsy.

Key words: absence epilepsy; spike-wave discharge; inbred strain; genetic; C3H; corticothalamic

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Received Dec 7, 2004; revised February 16, 2005; accepted February 18, 2005.

This article has been cited by other articles:

				L. A. Papale, B. Beyer, J. M. Jones, L. M. Sharkey, S. Tufik, M. Epstein, V. A. Letts, M. H. Meisler, W. N. Frankel, and A. Escayg Heterozygous mutations of the voltage-gated sodium channel SCN8A are associated with spike-wave discharges and absence epilepsy in mice Hum. Mol. Genet., May 1, 2009; 18(9): 1633 - 1641. [Abstract] [Full Text] [PDF]

				B. Beyer, C. Deleuze, V. A. Letts, C. L. Mahaffey, R. M. Boumil, T. A. Lew, J. R. Huguenard, and W. N. Frankel Absence seizures in C3H/HeJ and knockout mice caused by mutation of the AMPA receptor subunit Gria4 Hum. Mol. Genet., June 15, 2008; 17(12): 1738 - 1749. [Abstract] [Full Text] [PDF]

				H. O. Tan, C. A. Reid, F. N. Single, P. J. Davies, C. Chiu, S. Murphy, A. L. Clarke, L. Dibbens, H. Krestel, J. C. Mulley, et al. Reduced cortical inhibition in a mouse model of familial childhood absence epilepsy PNAS, October 30, 2007; 104(44): 17536 - 17541. [Abstract] [Full Text] [PDF]

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