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Allele Symbol Allele Name Allele ID |
Tg(Eno2-ATN1)3Tx transgene insertion 3, Tian Xu MGI:4360669 |
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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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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• reduced life span
• early-onset Sodium Butyrate (SB, a HDAC inhibitor) treatment (from 4 weeks old) extends their average life span (13.7%)
• late-onset (from 12 weeks old) SB treatment extends the average life span (21.5%)
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• neurons in the dentate cerebellar nucleus display somal atrophy
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• extensive nuclear accumulation of Atrophin-1 and widespread nuclear inclusions in several brain regions (striatum, cortex, thalamus, red nucleus, dentate cerebellar nucleus, and Purkinje cells)
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• progressively lose the capability to coordinate and balance in the rotarod test and the hanging wire test
• early-onset Sodium Butyrate (SB, a HDAC inhibitor) treatment (from 4 weeks old) delays the onset of motor impairments
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| dentatorubral-pallidoluysian atrophy | DOID:0060162 |
OMIM:125370 |
J:112706 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• no neurodegeneration
• much weaker nuclear accumulation and nearly no inclusions
• normal life span (>60 week)
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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 04/16/2024 MGI 6.23 |
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