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Phenotypes Associated with This Genotype
Genotype
MGI:6120563
Allelic
Composition
Gt(ROSA)26Sortm1.1(DUX4*)Plj/Gt(ROSA)26Sor+
Ndor1Tg(UBC-cre/ERT2)1Ejb/0
Genetic
Background
involves: 129S/SvEv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1.1(DUX4*)Plj mutation (1 available); any Gt(ROSA)26Sor mutation (1062 available)
Ndor1Tg(UBC-cre/ERT2)1Ejb mutation (11 available); any Ndor1 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• pups lose the righting reflex between 5-12 weeks of age
• between 5-12 weeks of age pups develop tremors
• between 5-12 weeks of age pups develop progressive ataxia
• pups develop spontaneous seizures characterized by periods of wild and uncontrolled running by 12 weeks of age

immune system
• swollen subiliac lymph nodes

integument
• P10 pups exhibit mild alopecia by 3 weeks, progressing to severe alopecia by 12 weeks of age
• pups exhibit delayed fur growth at P10

nervous system
• pups develop spontaneous seizures characterized by periods of wild and uncontrolled running by 12 weeks of age

skeleton
• between 5-12 weeks of age pups develop severe kyphosis


Contributing Projects:
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
01/06/2026
MGI 6.24
The Jackson Laboratory