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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp)4053Sbp
transgene insertion 4053, Stanley B Prusiner
MGI:5491029
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp)4053Sbp/0
involves: 129S7/SvEvBrd * FVB/N MGI:5491047
tg2
Tg(Prnp)4053Sbp/0 involves: FVB/N MGI:5491046


Genotype
MGI:5491047
cx1
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp)4053Sbp/0
Genetic
Background
involves: 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe mutation (39 available); any Prnp mutation (150 available)
Tg(Prnp)4053Sbp mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• mutants show increased susceptibility to developing scrapie following RML prion inoculation, with an average incubation time of 59 days compared to 138 days in controls and average death of 65 days versus 159 days in controls
• however, scrapie incubation times and time of death in mutants inoculated with RML prions is prolonged by about 10 days compared to single Tg(Prnp)4053Sbp mice




Genotype
MGI:5491046
tg2
Allelic
Composition
Tg(Prnp)4053Sbp/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mice remain free of central nervous system (CNS) dysfunction for more than 600 days of age, however 6 of 20 mutants develop CNS dysfunction between 605 and 815 days of age
• no neuropathic evidence for prion disease is evident

immune system
• mutants show increased susceptibility to developing scrapie following RML prion inoculation, with abbreviated incubation times (45 days vs. 138 days in controls) and earlier onset of death (58 days vs. 159 days) than controls
• however, brains of ill mutants inoculated with RML prions show reduced spongiform degeneration and reactive astrocytic gliosis compared to nontransgenic mice





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last database update
05/07/2024
MGI 6.23
The Jackson Laboratory