Phenotypes Associated with This Genotype

Genotype
MGI:5491044

• Allelic Composition: Tg(Prnp*P101L)2862Sbp/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 )

• abundant prion protein amyloid plaques

astrocytosis ( J:136434 )

• severe reactive astrocytic gliosis

spongiform encephalopathy ( J:136434 )

• severe spongiform degeneration

• vacuolation of the globus pallidus, cerebellum, substantia nigra, neocortex, and caudate is extensive

abnormal nervous system physiology ( J:136434 )

• 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