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Phenotypes Associated with This Genotype
Genotype
MGI:5518618
Allelic
Composition
Tg(Pcp2-ATXN1*82Q)5Horr/0
Genetic
Background
involves: FVB/N
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
• develop ataxia by 12 weeks of age (J:28932)
• develop ataxia by 12 weeks of age (J:28932)
• mice show a gentle swaying of the head while walking and early signs of general incoordination in the first 8-10 weeks of life, which progresses to ataxia (J:28932)
• mice show a gentle swaying of the head while walking and early signs of general incoordination in the first 8-10 weeks of life, which progresses to ataxia (J:28932)
• 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 (J:166951)
• 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 (J:166951)
• slight reduction in cage activity (J:28932)
• slight reduction in cage activity (J:28932)
• wider hind stance that controls (J:166951)
• wider hind stance that controls (J:166951)

nervous system
• cerebellar abnormalities (J:28932)
• cerebellar abnormalities (J:28932)
• year old mutants show severe atrophy and loss of Purkinje cells (J:166951)
• year old mutants show severe atrophy and loss of Purkinje cells (J:166951)
• atrophy of the finer dendritic branches of Purkinje cells (J:166951)
• atrophy of the finer dendritic branches of Purkinje cells (J:166951)
• reduction in molecular layer thickness (J:166951)
• reduction in molecular layer thickness (J:166951)
• mean relative height of climbing fiber terminals among Purkinje cell dendrites is reduced compared to wild-type mice, indicating compromised extension of climbing fiber terminals on Purkinje cells dendrites into the molecular layer (J:166951)
• mean relative height of climbing fiber terminals among Purkinje cell dendrites is reduced compared to wild-type mice, indicating compromised extension of climbing fiber terminals on Purkinje cells dendrites into the molecular layer (J:166951)

Mouse Models of Human Disease
OMIM ID Ref(s)
Spinocerebellar Ataxia 1; SCA1 164400 J:166951


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory