Phenotypes associated with this allele

• Allele Symbol: Tg(Slc32a1-cre)65Kzy
• Allele Name: transgene insertion 65, Kazuhiro Yamakawa
• Allele ID: MGI:5523786
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Scn1a^tm2.1Kzy/Scn1a^tm2.1Kzy Tg(Slc32a1-cre)65Kzy/0	involves: 129P2/OlaHsd * C57BL/6	MGI:5523884
cn2	Scn1a^tm2.1Kzy/Scn1a^+ Tg(Slc32a1-cre)65Kzy/0	involves: 129P2/OlaHsd * C57BL/6	MGI:5523885
cn3	Emx1^tm1.1(cre)Ito/Emx1^+ Scn1a^tm2.1Kzy/Scn1a^tm2.1Kzy Tg(Slc32a1-cre)65Kzy/0	involves: 129P2/OlaHsd * C57BL/6	MGI:5523887

Genotype
MGI:5523884

cn1

• Allelic Composition: Scn1a^tm2.1Kzy/Scn1a^tm2.1Kzy; Tg(Slc32a1-cre)65Kzy/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

postnatal lethality, complete penetrance ( J:202863 )

• all mice die by P15; average lifespan is 12.8 days

behavior/neurological

decreased locomotor activity ( J:202863 )

• mice are normal until P10, then behavioral hypoactivity is observed, except for myoclonic-like jerky movements

convulsive seizures ( J:202863 )

nervous system

convulsive seizures ( J:202863 )

abnormal hippocampus neuron morphology ( J:202863 )

• at P12.5, almost all Scn1a +ve (Nav1.1) neurons are almost undetectable

abnormal neocortex morphology ( J:202863 )

• at P12.5, almost all Scn1a +ve (Nav1.1) neurons are almost undetectable

abnormal cerebellar molecular layer ( J:202863 )

• at P12.5, almost all Scn1a +ve (Nav1.1) fibers are almost absent in the inner molecular layer of the cerebellum; however only a slight decrease is observed at the boundary between Purkinje and granule cell layers

Genotype
MGI:5523885

cn2

• Allelic Composition: Scn1a^tm2.1Kzy/Scn1a⁺; Tg(Slc32a1-cre)65Kzy/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

mortality/aging

premature death ( J:202863 )

• only 1 animal (1/65) lived to P35; rate is much higher than heterozygous Scn1a^tm2.2Kzy animals

postnatal lethality ( J:202863 )

• seizures occasionally result in immediate death

behavior/neurological

convulsive seizures ( J:202863 )

• mice are physically normal during first 2 weeks postnatal, but around P16 develop spontaneous generalized convulsive seizures

nervous system

convulsive seizures ( J:202863 )

• mice are physically normal during first 2 weeks postnatal, but around P16 develop spontaneous generalized convulsive seizures

abnormal brain wave pattern ( J:202863 )

• in cases of lethal seizures, postictal eletrocorticography (ECoG) activity progressively decreases and is undetectable within one minute after the seizure ends; if activity is maintained until recovery from postictal immobility and electrocorticography suppression, death does not result

Genotype
MGI:5523887

cn3

• Allelic Composition: Emx1^{tm1.1(cre)Ito}/Emx1⁺; Scn1a^tm2.1Kzy/Scn1a^tm2.1Kzy; Tg(Slc32a1-cre)65Kzy/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

mortality/aging

premature death ( J:202863 )

• mortality rate before P35 is around 40%, much lower than Scn1a^tm2.1Kzy/+ /Tg(Slc32a1-cre)65Kzy littermates (98%; 40/41)

behavior/neurological

convulsive seizures ( J:202863 )

• mice are normal through week 2 postnatally then develop convulsive seizures in the third week

nervous system

convulsive seizures ( J:202863 )

• mice are normal through week 2 postnatally then develop convulsive seizures in the third week