Audiogenic Seizure in Fmr1 Knockout Mice
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Fragile X patients have a mutation in the Fmr1 gene in which the gene, (FMRP; fragile X mental retardation protein), is not expressed. FMRP is an RNA binding protein that plays a pivotal role in synaptic functioning by translational regulation of dendritic mRNAs.
Fmr1 knockout (KO) mice have an increased susceptibility to audiogenic seizures (Thomas et al., 2011; Veeraragavan et al., 2011; Osterweil et al., 2010). Moreover, Fmr1 KO mice are reported to exhibit deficits in learning and memory tests such as fear conditioning (Zhao et al., 2005; Hayashi et al., 2007; Guo et al., 2012; Gu et al., 2002). Overall, this animal model is considered to be a clinically translatable model of fragile X syndrome.
After evaluating locomotor activity, mice are placed into Audiogenic Seizure test chambers with attached alarms. After acclimating, animal behavior is recorded for a 2 minute alarm challenge, a 1 minute resting period and an additional 2 minute challenge.
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