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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)
No mouse lines available in IMSR.
See publication links below for author information.
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
10/15/2019
MGI 6.14
The Jackson Laboratory