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to a single gene locus and/or that environmental effects might play a role. This mode of inheritance was confirmed in F2 (F1 x F1) and backcross (F1 x control) generations. When F2 population was generated by breeding GAERS with Brown Norway rats, a polygenic inheritance of SWD-related phenotypes was shown and three quantitative trait loci were identified that could control different variables of SWDs (e.g., frequency, amplitude, duration). In this study, the age of the animals was found to be a major factor influencing the detection of genetic linkage to the various components of the SWDs. The development of two inbred strains from the same initial colony has appeared as a very powerful tool to study the possible mutations involved in a genetically complex idiopathic epilepsy. A functional mutation in the
509:, was found using the two strains. In addition, the effect is due to a gain-of-function splice variant mutation, and is semi-dominant, explaining about 20% of the phenotypic variance in the cross. In heterologous expression studies, it was shown that the GAERS splice variant allele on Cav3.2 conferred faster recovery from channel inactivation and greater charge transference during high-frequency bursts. This is in agreement with a previous study that showed a selective increase in the T-type conductance in GAERS nRT neurons. It is also in line with the role of the low voltage activated Ca2+ channel in thalamic burst firing and genetic data in human patients.
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238:(EE) recordings. These seizures were recorded on both sides of the brain, lasted about 20 seconds and occurred when the animals were quiet. Importantly, SWDs were always associated with a typical "arrest" of the rats' behavior with twitching of the vibrissae. In addition, drugs used in the clinic to stop absence seizures (ethosuccimide,
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Powell, K.L., Cain, S.M., Ng, C., Sirdesai, S., David, L.S., Kyi, M., Garcia, E., Tyson, J.R., Reid, C.A., Bahlo, M., Foote, S.J., Snutch, T.P., O'Brien, T.J., 2009. A Cav3.2 T-Type
Calcium Channel Point Mutation Has Splice-Variant-Specific Effects on Function and Segregates with Seizure Expression
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In GAERS x NEC F1 generation, more than 95% of the animals showed SWDs after six months, suggesting a dominant transmission. Similar SWDs were recorded in males and females, indicating that the transmission is autosomal. Inter-individual variability suggested that the inheritance of SWDs is not due
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Using different methodologies (EEG, local field potentials, intracellular electrophysiology, functional MRI) it was demonstrated that spike-and-waves discharges are initiated in the somatosensory cortex in GAERS, more precisely in the area that codes for information from the vibrissae (barrel
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These initial observations led to the development of two breeding colonies: (i) a fully inbred strain of rats, with 100% of animals displaying the EEG and behavioral characteristics of absence seizures, derived from an outbred Wistar colony and called the
Genetic Absence Epilepsy Rats from
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Rudolf, G., Bihoreau, M.T., Godfrey, R.F., Wilder, S.P., Cox, R.D., Lathrop, M., Marescaux, C., Gauguier, D., 2004. Polygenic control of idiopathic generalized epilepsy phenotypes in the genetic absence rats from
Strasbourg (GAERS). Epilepsia 45,
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Strasbourg (GAERS) (ii) a strain of non epileptic control animals selected from the same initial breeding colony of Wistar rats and called the Non
Epileptic Control or NEC. Since then, the GAERS has been recognized as a very predictive model for
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Vergnes, M., Marescaux, C., Micheletti, G., Reis, J., Depaulis, A., Rumbach, L., Warter, J.M., 1982. Spontaneous paroxysmal electroclinical patterns in rat: a model of generalized non-convulsive epilepsy. Neuroscience
Letters 33,
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Depaulis, A., David, O., Charpier, S., 2016. The genetic absence epilepsy rat from
Strasbourg as a model to decipher the neuronal and network mechanisms of generalized idiopathic epilepsies. Journal of Neuroscience Methods 260,
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Depaulis, A., David, O., Charpier, S., 2016. The genetic absence epilepsy rat from
Strasbourg as a model to decipher the neuronal and network mechanisms of generalized idiopathic epilepsies. Journal of Neuroscience Methods 260,
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Micheletti, G., Vergnes, M., Marescaux, C., Reis, J., Depaulis, A., Rumbach, L., Warter, J.M., 1985. Antiepileptic drug evaluation in a new animal model: spontaneous petit mal epilepsy in the rat. Arzneimittelforschung 35,
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Micheletti, G., Vergnes, M., Marescaux, C., Reis, J., Depaulis, A., Rumbach, L., Warter, J.M., 1985. Antiepileptic drug evaluation in a new animal model: spontaneous petit mal epilepsy in the rat. Arzneimittelforschung 35,
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Micheletti, G., Vergnes, M., Marescaux, C., Reis, J., Depaulis, A., Rumbach, L., Warter, J.M., 1985. Antiepileptic drug evaluation in a new animal model: spontaneous petit mal epilepsy in the rat. Arzneimittelforschung 35,
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Micheletti, G., Vergnes, M., Marescaux, C., Reis, J., Depaulis, A., Rumbach, L., Warter, J.M., 1985. Antiepileptic drug evaluation in a new animal model: spontaneous petit mal epilepsy in the rat. Arzneimittelforschung 35,
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Micheletti, G., Vergnes, M., Marescaux, C., Reis, J., Depaulis, A., Rumbach, L., Warter, J.M., 1985. Antiepileptic drug evaluation in a new animal model: spontaneous petit mal epilepsy in the rat. Arzneimittelforschung 35,
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Micheletti, G., Vergnes, M., Marescaux, C., Reis, J., Depaulis, A., Rumbach, L., Warter, J.M., 1985. Antiepileptic drug evaluation in a new animal model: spontaneous petit mal epilepsy in the rat. Arzneimittelforschung 35,
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Tsakiridou, E., Bertollini, L., deCurtis, M., Avanzini, G., Pape, H.C., 1995. Selective increase in T-Type calcium conductance of reticular thalamic neurons in a rat model of absence epilepsy. Journal of
Neuroscience 15,
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Depaulis, A., van
Luijtelaar, G., 2005. Genetic models of absence epilepsy in the rat, in: Pitkänen, A., Schwartzkroin, P., Moshe, S. (Eds.), Models of Seizures and Epilepsy. Oxford: Elsevier Academic, Amsterdam, pp.
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In GAERS, absence epilepsy develops during the cortical maturation, i.e., the first 3–4 weeks after birth. Abnormal oscillations are EEG recorded in GAERS at postnatal day (P) 15. They progressively evolve into
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Gower, A.J., Hirsch, E., Boehrer, A., Noyer, M., Marescaux, C., 1995. Effects of levetiracetam, a novel antiepileptic drug, on convulsant activity in two genetic rat models of epilepsy. Epilepsy
Research 22,
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Vartanian, M.G., Radulovic, L.L., Kinsora, J.J., Serpa, K.A., Vergnes, M., Bertram, E., Taylor, C.P., 2006. Activity profile of pregabalin in rodent models of epilepsy and ataxia. Epilepsy Research 68,
283:, along with the WAG/Rij rat model. The colony, initially developed in Strasbourg, is maintained at the University of Grenoble Alpes, under Inserm licence and the supervision of Antoine Depaulis.
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cortex). Using intracellular electrophysiological recordings of the different layers of the somatosensory cortex, it was found that pyramidal cell of the deep layer (L5/6) initiate the spikes
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Polack, P.-O. et al. Deep layer somatosensory cortical neurons initiate spike-and-wave discharges in a genetic model of absence seizures. The Journal of Neuroscience 27, 6590–6599 (2007).
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Liu, Z., Seiler, N., Marescaux, C., Depaulis, A., Vergnes, M., 1990. Potentiation of gamma-vinyl GABA (vigabatrin) effects by glycine. European Journal of Pharmacology 182, 109–115.
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Danober, L., Deransart, C., Depaulis, A., Vergnes, M., Marescaux, C., 1998. Pathophysiological mechanisms of genetic absence epilepsy in the rat. Prog. Neurobiol. 55, 27–57
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Danober, L., Deransart, C., Depaulis, A., Vergnes, M., Marescaux, C., 1998. Pathophysiological mechanisms of genetic absence epilepsy in the rat. Prog. Neurobiol. 55, 27–57
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Danober, L., Deransart, C., Depaulis, A., Vergnes, M., Marescaux, C., 1998. Pathophysiological mechanisms of genetic absence epilepsy in the rat. Prog. Neurobiol. 55, 27–57
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Marescaux, C., Vergnes, M., Depaulis, A., 1992. Genetic absence epilepsy in rats from Strasbourg--a review. Journal of Neural Transmission - Supplement 35, 37–69.
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Jarre, G. et al. Building Up Absence Seizures in the Somatosensory Cortex: From Network to Cellular Epileptogenic Processes. Cerebral Cortex 27, 4607–4623 (2017)
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Marescaux, C., Vergnes, M., Depaulis, A., 1992. Genetic absence epilepsy in rats from Strasbourg--a review. Journal of Neural Transmission - Supplement 35, 37–69
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Marescaux, C., Vergnes, M., Depaulis, A., 1992. Genetic absence epilepsy in rats from Strasbourg--a review. Journal of Neural Transmission - Supplement 35, 37–69
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characterized by recurrent loss of contact and concomitant pattern on the electroencephalogram called "spike-and-wave" discharges. It was first characterized in
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The reactivity of GAERS to antiepileptic drugs is unique since it perfectly matches with the effects of these drugs in patients with typical absence epilepsy
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Augusto Grinspan, Evaluación de la eficacia de la lamotrigine en agudo en las GAERS, PhD dissertation, Buenos aires, 1995
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in a Polygenic Rat Model of Absence Epilepsy. Journal of Neuroscience 29, 371–380.
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The following table summarizes the effects of the different
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Hughes, J.R., 2009. Epilepsy & Behavior. 15, 404–412.
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