Mouse Genome Informatics
ht
    Scn1btm1.1Bion/Scn1b+
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• when exposed to a stream of warm air, mice exhibit febrile seizures (including behavioral arrest, facial automatisms, and early epileptiform discharges localized to the hippocampus) unlike similarly treated wild-type mice
• mice exhibit febrile seizures at lower temperatures than wild-type mice
• average burst duration in subicular pyramidal neurons is lengthened compared to in wild-type cells
• neurons exhibit axonal hyperexcitability, increased spike gain, and increased burst firing compared with wild-type cells
• however, total burst events and frequency of action potentials within bursts is normal
• the number of action potentials (APs) per bursts is increased compared to in wild-type mice
• neurons begin firing APs at lower drive currents compared to in wild-type mice
• the threshold for AP initiation is more negative than in wild-type cells
• the amplitude of first APs is higher than in wild-type cells
• the AP half-width is decreased compared to in wild-type cells
• however, the frequency of action potentials within bursts is normal

behavior/neurological
• when exposed to a stream of warm air, mice exhibit febrile seizures (including behavioral arrest, facial automatisms, and early epileptiform discharges localized to the hippocampus) unlike similarly treated wild-type mice
• mice exhibit febrile seizures at lower temperatures than wild-type mice

Mouse Models of Human Disease
OMIM IDRef(s)
Generalized Epilepsy with Febrile Seizures Plus, Type 1; GEFSP1 604233 J:163771