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Phenotypes Associated with This Genotype
Genotype
MGI:5487546
Allelic
Composition
Tg(Prnp*P101L)174Sbp/0
Genetic
Background
involves: C57BL/6 * SJL
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No mouse lines available in IMSR.
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phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• once mice develop neurologic dysfunction, they deteriorate over 3-36 days until death

behavior/neurological
• ataxia and symptoms of rigidity develop between 7 and 39 weeks of age
• mice become lethargic between 7 and 39 weeks of age

nervous system
• mean age for when mutants develop neurologic dysfunction is 166 +/- 6 days; once affected, they deteriorate over 3-36 days until death
• however, amyloid plaques are not seen in the brain and brain homogenates from mutants are not infectious when injected into other mice or hamsters
• prominent Bergmann radial gliosis is seen in much of the molecular layer of the cerebellar cortex
• mild to moderate reactive astrocytic gliosis in a patchy distribution in both gray and white matter
• numerous vacuoles (spongiform degeneration) are present in both the gray and white matter of the cerebral hemispheres and brainstem

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Gerstmann-Straussler-Scheinker syndrome DOID:4249 OMIM:137440
J:127373


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
03/19/2019
MGI 6.13
The Jackson Laboratory