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Phenotypes Associated with This Genotype
Genotype
MGI:3836992
Allelic
Composition
Tg(Prnp*D177N*M128V)A21Rchi/Tg(Prnp*D177N*M128V)A21Rchi
Genetic
Background
involves: C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp*D177N*M128V)A21Rchi mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die from neurological disease with a mean lifespan of 284 days
• the length of illness (mean day of onset until death) is 130 days

behavior/neurological
• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail
• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 144 days
• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 144 days

nervous system
• protease resistant Prnp deposits are found in many parts of the brain in mice suffering from neurological disease
• Prnp deposits ranged from diffuse synaptic-type depositions prominent in the hippocampus to plaque-like depositions in the reticular thalamic nucleus, the corpus callosum, the external and internal capsule, the cingulated cortex, and the anterior commissure nuclei
• depositions do not contain amyloid fibrils
• hypertrophy and proliferation of astrocytes are prominent adjacent to Prnp depositions

skeleton
• 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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
08/09/2022
MGI 6.21
The Jackson Laboratory