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Phenotypes Associated with This Genotype
Genotype
MGI:5518629
Allelic
Composition
Tg(Pcp2-ATXN1*30Q*S776D)2Horr/0
Genetic
Background
Not Specified
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
behavior/neurological
• mutants are impaired on the accelerating rotarod at 6 and 12 weeks of age and by 30 weeks of age, are unable to perform the test
• wider hind stance that controls

nervous system
• Purkinje cells from 12 week old mutants show dendritic atrophy, especially of the finer dendritic branches
• however, Purkinje cell death is not observed
• reduction in molecular layer thickness
• thickness of the molecular layer and Purkinje cells at one year of age show little further atrophy from that seen at 12 weeks of age, indicating that the disease fails to advance to neuronal cell death
• mean relative height of climbing fiber terminals among Purkinje cell dendrites is reduced at 12 weeks of age compared to wild-type mice, indicating compromised extension of climbing fiber terminals on Purkinje cells dendrites into the molecular layer


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
03/25/2025
MGI 6.24
The Jackson Laboratory