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Phenotypes Associated with This Genotype
Genotype
MGI:5317102
Allelic
Composition
Tg(Pcp2-tTA)3Horr/0
Tg(tetO-ATXN1*82Q)#Horr/Tg(tetO-ATXN1*82Q)#Horr
Genetic
Background
involves: FVB/N
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Mouse lines carrying:
Tg(Pcp2-tTA)3Horr mutation (1 available)
Tg(tetO-ATXN1*82Q)#Horr mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• by 12 weeks of age, but not at 6 weeks of age, mutants start to show signs of ataxia by home cage behavior (J:95453)
• by 32 weeks of age, mutants exhibit overt ataxia (J:95453)
• treatment with doxycycline at 12 or at 32 weeks of age to turn off expression of the gene results in improvement in home cage behavior (J:95453)
• by 12 weeks of age, but not at 6 weeks of age, mutants start to show signs of ataxia by home cage behavior (J:95453)
• by 32 weeks of age, mutants exhibit overt ataxia (J:95453)
• treatment with doxycycline at 12 or at 32 weeks of age to turn off expression of the gene results in improvement in home cage behavior (J:95453)
• 6 week old mutants exhibit a moderate deficiency on the accelerating rotarod which gets worse at 12 weeks of age and by 32 weeks of age, mutants are unable to stay on the accelerating rotarod (J:95453)
• treatment with doxycycline at 6, 12 or at 32 weeks of age to turn off expression of the gene results in a partial improvement in rotarod performance, but only after an extended period of dox treatment (J:95453)
• 6 week old mutants exhibit a moderate deficiency on the accelerating rotarod which gets worse at 12 weeks of age and by 32 weeks of age, mutants are unable to stay on the accelerating rotarod (J:95453)
• treatment with doxycycline at 6, 12 or at 32 weeks of age to turn off expression of the gene results in a partial improvement in rotarod performance, but only after an extended period of dox treatment (J:95453)

nervous system
• 6 week old mutants exhibit a mild Purkinje cell atrophy that includes cytoplasmic vacuoles, some dendritic pruning, heterotypic Purkinje and nuclear inclusions (J:95453)
• by 12 and 32 weeks of age, Purkinje cell atrophy is very extensive (J:95453)
• treatment with doxycycline at 6, 12 or at 32 weeks of age to turn off expression of the gene results in improvement of Purkinje cell pathology (J:95453)
• 6 week old mutants exhibit a mild Purkinje cell atrophy that includes cytoplasmic vacuoles, some dendritic pruning, heterotypic Purkinje and nuclear inclusions (J:95453)
• by 12 and 32 weeks of age, Purkinje cell atrophy is very extensive (J:95453)
• treatment with doxycycline at 6, 12 or at 32 weeks of age to turn off expression of the gene results in improvement of Purkinje cell pathology (J:95453)
• at 12 weeks of age, mutants exhibit a decrease in dendritic arborization and distribution of spines along the remaining dendrites (J:95453)
• treatment with doxycycline at 12 or at 32 weeks of age to turn off expression of the gene results in increased arborization of the Purkinje cell dendritic tree (J:95453)
• at 12 weeks of age, mutants exhibit a decrease in dendritic arborization and distribution of spines along the remaining dendrites (J:95453)
• treatment with doxycycline at 12 or at 32 weeks of age to turn off expression of the gene results in increased arborization of the Purkinje cell dendritic tree (J:95453)

Mouse Models of Human Disease
OMIM ID Ref(s)
Spinocerebellar Ataxia 1; SCA1 164400 J:95453


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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory