About   Help   FAQ
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
DO ID OMIM ID(s) Ref(s)
spinocerebellar ataxia type 17 DOID:0050967 OMIM:607136
J:130775





Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
03/12/2024
MGI 6.23
The Jackson Laboratory