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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp*A116V*M128V)1309Jama
transgene insertion 1309, James A Mastrianni
MGI:4356192
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp*A116V*M128V)1309Jama/0
involves: 129S7/SvEvBrd MGI:4356193


Genotype
MGI:4356193
cx1
Allelic
Composition
Prnptm1Cwe/Prnptm1Cwe
Tg(Prnp*A116V*M128V)1309Jama/0
Genetic
Background
involves: 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe mutation (29 available); any Prnp mutation (89 available)
Tg(Prnp*A116V*M128V)1309Jama mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• at 176 days

behavior/neurological
• beginning at 5 months, mice display a widened stance and unsteady gait compared with wild-type mice
• ataxia progresses with time and becomes severe with excessive falling, difficulty righting, and death
• beginning at 5 months, mice display a widened stance and unsteady gait compared with wild-type mice

nervous system
• mice develop amyloid plaques positive for full-length Prnp protein in the neocortex, hippocampus, caudate nucleus, and cerebellar cortex
• mice exhibit mild scattered vacuolization in the neocortex, hippocampus, thalamus, hypothalamus, caudate nucleus, pons, cerebellum molecular layer, and cerebellum granular layer

homeostasis/metabolism
• mice develop amyloid plaques positive for full-length Prnp protein in the neocortex, hippocampus, caudate nucleus, and cerebellar cortex

Mouse Models of Human Disease
OMIM ID Ref(s)
Gerstmann-Straussler Disease; GSD 137440 J:151934





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last database update
08/23/2016
MGI 6.05
The Jackson Laboratory