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Phenotypes Associated with This Genotype
Genotype
MGI:5491044
Allelic
Composition
Tg(Prnp*P101L)2862Sbp/0
Genetic
Background
involves: FVB/N
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phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mutants begin to exhibit ataxia and rigidity between 150 and 200 days of age
• mutants begin to exhibit lethargy between 150 and 200 days of age

nervous system
• abundant prion protein amyloid plaques
• severe reactive astrocytic gliosis
• severe spongiform degeneration
• vacuolation of the globus pallidus, cerebellum, substantia nigra, neocortex, and caudate is extensive
• 100% of mutants develop central nervous system dysfunction by about 410 days of age
• mean age of disease onset is 321 +/- 21 days

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Gerstmann-Straussler-Scheinker syndrome DOID:4249 OMIM:137440
J:136434


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/05/2019
MGI 6.13
The Jackson Laboratory