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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-TBP*)105Xjl
transgene insertion 105, Xiao-Jiang Li
MGI:3772700
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Prnp-TBP*)105Xjl/0 FVB/N-Tg(Prnp-TBP*)105Xjl MGI:3772885


Genotype
MGI:3772885
tg1
Allelic
Composition
Tg(Prnp-TBP*)105Xjl/0
Genetic
Background
FVB/N-Tg(Prnp-TBP*)105Xjl
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-TBP*)105Xjl 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 as early as 9 weeks of age

growth/size/body
• symptomatic mice are visibly smaller than normal littermates at 3 months
• mice begin to lose body weight at 8 weeks of age

behavior/neurological
• symptomatic mice can be distinguished from normal littermates at 3 months of age by poorly groomed appearance
• displayed by some mice
• displayed by some mice
• onset of progressive motor impairment is 6 weeks of age
• mice perform poorly on a non-accelerating rotating rod at 6 weeks of age, and do not show any subsequent improvement
• some mice exhibit spontaneous seizures

nervous system
• some mice exhibit spontaneous seizures
• gliosis is observed in granular and Purkinje cell layers of cerebellum
• loss or disruption of calbindin-positive neurites in cerebellar molecular layer is observed in mutants
• degenerating neurons are detected in granular layer of cerebellum
• degenerating Purkinje cells are evident in cerebellum
• prominent nuclear inclusions form in cerebellar granule neurons
• 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

skeleton
• posture is conspicuously abnormal in mutants; kyphosis, indicative of proximal muscle weakness, is observed by 3 months of age

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





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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory