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Phenotypes Associated with This Genotype
Genotype
MGI:4360690
Allelic
Composition
Tg(Eno2-ATN1)3Tx/Tg(Eno2-ATN1)3Tx
Genetic
Background
B6.Cg-Tg(Eno2-ATN1)3Tx
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 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%)

nervous system
• neurons in the dentate cerebellar nucleus display somal atrophy
• 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)

behavior/neurological
• 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

Mouse Models of Human Disease
OMIM ID Ref(s)
Dentatorubral-Pallidoluysian Atrophy; DRPLA 125370 J:112706


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
08/23/2016
MGI 6.05
The Jackson Laboratory