Phenotypes Associated with This Genotype

Genotype
MGI:5428333

• Allelic Composition: Prnp^tm1Cwe/Prnp^tm1Cwe; Tg(Prnp*)#Rgab/0
• Genetic Background: involves: 129S7/SvEvBrd * C57BL/6 * FVB/N

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

mortality/aging

premature death ( J:183170 )

• mice are euthanized when they are too paralyzed to reach food and water

behavior/neurological

limb grasping ( J:183170 )

tremors ( J:183170 )

• in very few mice

hindlimb paralysis ( J:183170 )

• asymmetric hindlimb weakness at 5 to 6 months that progresses to paraplegia

paraparesis ( J:183170 )

• asymmetric hindlimb weakness at 5 to 6 months that progresses to paraplegia

myoclonus ( J:183170 )

• myoclonic jerks in some mice

limbs/digits/tail

abnormal tail morphology ( J:183170 )

• plastic tail in very few mice

muscle

myoclonus ( J:183170 )

• myoclonic jerks in some mice

muscular atrophy ( J:183170 )

• in the lower body

nervous system

myoclonus ( J:183170 )

• myoclonic jerks in some mice

astrocytosis ( J:183170 )

• at 3 months and more prominent at 8 months

neuron degeneration ( J:183170 )

• at 3 months and more prominent at 8 months

skeleton

kyphosis ( J:183170 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Creutzfeldt-Jakob disease		DOID:11949	OMIM:123400	J:183170