Analysis Tools|
Allele Symbol Allele Name Allele ID |
Tg(Scn1a*)RH9Aesc transgene insertion RH9, Andrew Escayg MGI:3851210 |
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| Summary |
3 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• a few mice survive beyond weaning but mice rarely survive past 6 months of age
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• unlike homozygous mice not carrying the transgene which all die by P15, most mice carrying the transgene die between P17 and P21
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• substantial improvement in life span compared to heterozygous mice not carrying the transgene; 50% survival at 6 months of age compared to 25%
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• decreased threshold for kainic acid induced seizures compared to control transgenic lines
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• the ratio of sodium current densities in bipolar versus pyramidal neurons is reduced and the sodium current density in bipolar cells is reduced compared to controls
• sodium channels appear to recover more slowly from inactivation and current through the channels decreases during a series of depolarizations in bipolar neurons
• in pyramidal neurons a negative shift is seen in the voltage dependence of inactivation
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• decreased threshold for kainic acid induced seizures compared to control transgenic lines
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| generalized epilepsy with febrile seizures plus | DOID:0060170 | J:150454 | ||
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/19/2024 MGI 6.24 |
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