A Review on Genetic Mutant Mouse Models in Epilepsy Studies

Animal models that are used in epilepsy studies must be able to show spike and wave discharge (SWDs) activity in the cortex and thalamus as the land mark of epilepsy in human form. There are several epileptic mutants mouse models such as tottering, lethargic, stargazer, mocha, slow-wave-epilepsy and ducky. Tottering mice are the best model for ataxia. The manifestation of tottering seizures is same ashuman. The frequency of SWDs in these mice is higher (6-7 Hz) than human. The phenotype of lethargic mouse appears after 15 days of age by ataxia and lethargic behavior accompanied to focal motor seizures. Generalized cortical SWDs are the second seizure type which is recorded in their EEG. The characteristic of their seizures is the same as human and tottering seizures. The phenotype of stargazer is similar to the other mutant mice including ataxia, paroxysmal dyskinesia and head tossing. The characteristic of seizures in mocha is same as stargazer mouse. The frequency of SWDs is 6-7 Hz and phenotype accompanied to ataxia, tonic-clonic seizures, pigment dilution, and increased bleeding time. SWDs in slow-wave-epilepsy mouse appear with frequency of 3-4.5 Hz during seizures. It has been considered as a valid model for epilepsy due to seizure phenotype and anti-epileptic-drug responses. This exchanger regulates the hemostasis, cell volumeand mitogenic responses to growth factors. Ataxia, tonicclonic seizures and cerebellum degeneration are somephenotype appeared in these mutants beside of absence seizures. The frequency of SWDs in ducky mice same as the most of mice model of epilepsy is 6 Hz. Ataxia,limb dyskinesia, and developmental dysgenesis of some brain areas like cerebellum, medulla and spinal cord are some additional phenotypes that exist in these mice. Conclusion: In conclusion, epileptic mutants mouse are favored models in neurobiology and neuropharmacologyfor the study of abnormal brain synchronization, but they have pioneer role in the neurogenic analysis of seizure disorders and their number are restricted by the rate of mutations, feasible dysfunctions. Also, in the mutant mousses models generally a single gene underlie the epileptic phenotype (SWDs), however, in humans a polygenetic mode of inheritance occurs.