Phenotypes associated with this allele

• Allele Symbol: Tg(Prnp*P101L)2247Sbp
• Allele Name: transgene insertion 2247, Stanley B Prusiner
• Allele ID: MGI:5491023
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(Prnp*P101L)2247Sbp/0	involves: FVB/N	MGI:5491030

Genotype
MGI:5491030

tg1

• Allelic Composition: Tg(Prnp*P101L)2247Sbp/0
• Genetic Background: involves: FVB/N

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

behavior/neurological

lethargy ( J:136434 )

• mutants begin to exhibit lethargy between 150 and 200 days of age

ataxia ( J:136434 )

• mutants begin to exhibit ataxia and rigidity between 150 and 200 days of age

nervous system

abnormal brain morphology ( J:136434 )

• occasional prion protein plaques are seen in brains of a few ill mutants

astrocytosis ( J:136434 )

• reactive astrocytic gliosis

neurodegeneration ( J:136434 )

• mutants show neurodegeneration between 150 and 300 days of age

spongiform encephalopathy ( J:136434 )

abnormal nervous system physiology ( J:136434 )

• 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