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Phenotypes Associated with This Genotype
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
• mice have reduced lifespans relative to wild-type; mice start to die as early as 9 weeks of age

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

• 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

• 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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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.01
The Jackson Laboratory