Mouse Genome Informatics
cn
    Scn1atm2.1Wac/Scn1a+
Tg(I12b-cre)1Jlr/0

involves: C57BL/6J * CD-1
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mice begin to die in the third postnatal week
• only 30% of mice survive to 90 days of age
• mice die 4-48 h after initial seizure onset

behavior/neurological
• spontaneous seizures are seen in about 1/3 of mice
• mice die 4-48 h after initial seizure onset
• elevation of body temperature induces seizure (mean temperature for seizure induction: 39.6 oC)
• seizures begin with forelimb clonus and progress in both frequency and severity through forelimb clonus with rearing
• seizures eventually culminate in generalized tonic-clonic seizure

nervous system
• spontaneous seizures are seen in about 1/3 of mice
• mice die 4-48 h after initial seizure onset
• elevation of body temperature induces seizure (mean temperature for seizure induction: 39.6 oC)
• seizures begin with forelimb clonus and progress in both frequency and severity through forelimb clonus with rearing
• seizures eventually culminate in generalized tonic-clonic seizure

Mouse Models of Human Disease
OMIM IDRef(s)
Epileptic Encephalopathy, Early Infantile, 6; EIEE6 607208 J:189897