Analysis Tools
mortality/aging
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• less than 3% of the expected 25% of mutant pups are born and those pups then die immediately
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Allele Symbol Allele Name Allele ID |
Gt(ROSA)26Sortm1(DUX4)Sqh targeted mutation 1, Scott Harper MGI:6273758 |
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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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• less than 3% of the expected 25% of mutant pups are born and those pups then die immediately
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in smaller fiber diameters
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• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in diaphragms from 6 out of 18 mice that exhibit myofibers with greater than 10% central nuclei
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• high dose (150 mg/kg) administration of tamoxifen by oral gavage results in severe muscle degeneration in skeletal muscle within 7-9 days
• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in damage to tibialis anterior, gastrocnemius, quadriceps and triceps within 4 weeks
• at 0.5mg/kg tamoxifen administration results in a decrease in mild sporadic lesions beginning at 1 month, with a dramatic increase in severity by 3 and 4 months
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• TA muscles in uninduced 1.5 year old mice are 33% smaller than controls with reduced absolute force output
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• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in muscle weakness
• TA muscles in uninduced 1.5 year old mice develop reduced absolute force output
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• high dose (150 mg/kg) administration of tamoxifen by oral gavage results in immune cell infiltrates in skeletal muscle within 7-9 days
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• administration of tamoxifen (high dose - 150 mg/kg) by oral gavage results in a slow, unsteady gait, by 10 days mice are non-ambulatory
• administration of tamoxifen (medium dose - 5 mg/kg) by oral gavage results in gait changes within 3 weeks
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• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in a reduction in rearing frequency
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• high dose (150 mg/kg) administration of tamoxifen by oral gavage results in a reduction in overall activity, by 10 days mice are non-ambulatory
• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in a reduction in total activity
• at 0.5mg/kg tamoxifen administration results in a decrease in activity beginning at 1 month, with significant reduction by 2 months, however mice recover to wild-type levels by 3-4 months
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| facioscapulohumeral muscular dystrophy | DOID:11727 | J:268959 | ||
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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 05/21/2024 MGI 6.23 |
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