Mouse Genome Informatics
tg
    Tg(Pcp2-ATXN1*82Q)5Horr/0
involves: FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• 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)
• 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
• wider hind stance that controls

nervous system
• cerebellar abnormalities
• year old mutants show severe atrophy and loss of Purkinje cells
• atrophy of the finer dendritic branches of Purkinje cells
• reduction in molecular layer thickness
• 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

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