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Phenotypes Associated with This Genotype
Genotype
MGI:3772883
Allelic
Composition
Tg(Prnp-TBP*)71-16Xjl/0
Genetic
Background
FVB/N-Tg(Prnp-TBP*)71-16Xjl
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TBP*)71-16Xjl mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice have reduced lifespans relative to wild-type; mice start to die at 21 weeks of age (J:130775)
• mice have reduced lifespans relative to wild-type; mice start to die at 21 weeks of age (J:130775)

growth/size/body
• symptomatic mice are visibly smaller than normal littermates at 6.5 months (J:130775)
• symptomatic mice are visibly smaller than normal littermates at 6.5 months (J:130775)

behavior/neurological
• symptomatic mice can be distinguished from normal littermates at 6.5 months of age by poorly groomed appearance (J:130775)
• symptomatic mice can be distinguished from normal littermates at 6.5 months of age by poorly groomed appearance (J:130775)
• displayed by some mice (J:130775)
• displayed by some mice (J:130775)
• displayed by some mice (J:130775)
• displayed by some mice (J:130775)
• onset of progressive motor impairment is 7 weeks of age (J:130775)
• onset of progressive motor impairment is 7 weeks of age (J:130775)
• mice show better initial performance relative to the other transgenic lines, then show a steady decline in performance (J:130775)
• mice show better initial performance relative to the other transgenic lines, then show a steady decline in performance (J:130775)
• decreased rotarod performance (J:221703)
• decreased rotarod performance (J:221703)
• some mice exhibit spontaneous seizures (J:130775)
• some mice exhibit spontaneous seizures (J:130775)

nervous system
• some mice exhibit spontaneous seizures (J:130775)
• some mice exhibit spontaneous seizures (J:130775)
• gliosis is observed in granular and Purkinje cell layers of cerebellum (J:130775)
• gliosis is observed in granular and Purkinje cell layers of cerebellum (J:130775)
• TBP-71Q is relatively diffuse in neuronal nuclei in the brain (J:130775)
• TBP-71Q is relatively diffuse in neuronal nuclei in the brain (J:130775)
• degenerating Purkinje cells are evident in cerebellum (J:130775)
• degenerating Purkinje cells are evident in cerebellum (J:130775)
• loss or disruption of calbindin-positive neurites in cerebellar molecular layer is observed in mutants (J:130775)
• loss or disruption of calbindin-positive neurites in cerebellar molecular layer is observed in mutants (J:130775)
• degenerating axons are evident in cerebellum; axons with reduced internal space surrounded by a distorted or thickened myelin sheath, presence of myelin ovoids, or vacuolated axons without distinguishable organelles or disintegrating myelin sheaths are indicative of more severe degeneration (J:130775)
• degenerating axons are evident in cerebellum; axons with reduced internal space surrounded by a distorted or thickened myelin sheath, presence of myelin ovoids, or vacuolated axons without distinguishable organelles or disintegrating myelin sheaths are indicative of more severe degeneration (J:130775)

Mouse Models of Human Disease
OMIM ID Ref(s)
Spinocerebellar Ataxia 17; SCA17 607136 J:130775


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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