Phenotypes Associated with This Genotype

Genotype
MGI:3836994

• Allelic Composition: Prnp^tm1Cwe/Prnp^tm1Cwe; Tg(Prnp*D177N*M128V)A21Rchi/Tg(Prnp*D177N*M128V)A21Rchi
• Genetic Background: involves: 129S7/SvEvBrd * C57BL/6 * CBA

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

mortality/aging

premature death ( J:142098 )

• mice die from neurological disease with a mean lifespan of around 284 days

• the length of illness (mean day of onset until death) is around 130 days

behavior/neurological

limb grasping ( J:142098 )

• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail

ataxia ( J:142098 )

• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset around 144 days

impaired balance ( J:142098 )

• mice develop ataxia with extension of hindlimbs and the unbalanced posture with an age of onset around 144 days

skeleton

kyphosis ( J:142098 )

• kyphosis occurs with the onset of ataxia

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Creutzfeldt-Jakob disease		DOID:11949	OMIM:123400	J:142098