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Phenotypes Associated with This Genotype
Genotype
MGI:5491030
Allelic
Composition
Tg(Prnp*P101L)2247Sbp/0
Genetic
Background
involves: FVB/N
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See publication links below for author information.
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
• occasional prion protein plaques are seen in brains of a few ill mutants
• reactive astrocytic gliosis
• mutants show neurodegeneration between 150 and 300 days of age
• 100% of mutants develop central nervous system dysfunction by 300 days of age
• mean age of disease onset is 227 +/- 16 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 Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
10/08/2019
MGI 6.14
The Jackson Laboratory