Transcription profiling of mouse brain and cerebellum from adult mutant stg, tg, and lh mice, which display the ataxic and epileptic phenotypes vs. wild type litter-mate controls

ABSTRACT: Inherited mutations of calcium ion channels exhibit neurological defects, such as epilepsy, ataxia, and migraine, and these phenotypes are shared among humans and mouse models. Absence epilepsy and ataxic phenotypes are present in the calcium channelopathy mutants stargazer (stg-gamma2 subunit), tottering (tg-alpha1 subunit), and lethargic (lh-beta4 subunit). These mutations of high-voltage-activated (HVA) calcium channel subunits initiate increases in membrane excitability of low-voltage-activated (LVA) calcium channels in thalamic neurons, thus enhancing LVA currents. Elevated LVA currents, produced from T-type calcium channels, induce rhythmic thalamocortical burst firing and spike-wave seizures. The changes in gene expression originating from the different mutations result in simila

ORGANISM(S): Mus musculus

SUBMITTER: Elizabeth Salomon 

PROVIDER: E-GEOD-6275 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress